Daniel R. Pieroni

Echocardiographic detection of intracardiac right-to-left shunts following peripheral vein injections.

SUMMARY A contrast echocardiographic technique using peripheral vein injections for the detection and localization of intracardiac right-to-left shunting is described. Fifty children underwent cardiac catheterization and peripheral contrast echocardiographic studies. The site, degree, and direction of shunting were established at catheterization by oximetry, indicator dilution analysis and/or angiography. Peripheral vein injections were performed from the right antecubital vein or the right saphenous vein. Right-to-left shunts were documented at catheterization in 27/50 patients; contrast echocardiographic studies indicated the presence and level of shunting in all 27 patients. The contrast echocardiographic technique using peripheral vein injections detects and localizes right-to-left intracardiac shunting. It is a safe and sensitive method to evaluate systemic desaturation in ambulatory and postoperative patients.

Recognition of residual postoperative shunts by contrast echocardiographic techniques.

A bedside echocardiographic technique was used to detect and localize residual intracardiac shunts in 26 patients who had surgical repair of septal defects. Contrast echocardiography was performed through central venous and left atrial monitoring catheters at the same time as cardiogreen dye curves. Indicator dilution confirmed residual atrial defects in ten patients and ventricular defects in five. Contrast echocardiography indicated the presence and level of shunting in all 15 patients. Temporary flow through newly implanted septal patches was detected and differentiated from shunting across a true residual defect. The contrast echocardiographic technique using injections through the central venous and left atrial catheters as described detects and localizes right-to-left and left-to-right shunting. It is a safe and reliable method to evaluate residual intracardiac defects postoperatively.

Mycotic Aortic Aneurysms in Children

Mycotic aneurysms of the aorta are uncommon in babies and children. Prior to the development of antibiotics, most mycotic aneurysms were seen secondary to bacterial endocarditis, but this is now uncommon. Instead, more cases have been reported as complications of umbilical artery catheters in newborns. We have seen five cases of mycotic aneurysms in children, two of them secondary to umbilical artery catheters. One patient had coarctation of the aorta, and the other patients had different sources of infection. Three patients were treated surgically by us with good results after antibiotic therapy. One patient died of sepsis before the aneurysm was diagnosed. The fifth patient was treated elsewhere and now has a recurrent aneurysm. We think a combination of aggressive medical and early surgical therapy may save a high percentage of these patients.

Association of Kawasaki disease and interstitial nephritis

Renal insufficiency is a rare manifestation of Kawasaki disease. We report a 2·5-year-old boy with Kawasaki disease who developed acute renal failure during the acute phase of his illness. A percutaneous renal biopsy revealed acute interstitial nephritis. No etiological agent could be identified and renal recovery occurred with supportive care alone.

Two-dimensional echocardiographic examination of mitral valve abnormalities associated with coarctation of the aorta.

Coarctation of the aorta is frequently associated with left ventricular inflow tract abnormalities that may be difficult to detect even at cardiac catheterization. This study involved patients with coarctation who underwent comprehensive two-dimensional echocardiographic investigations emphasizing visualization of the mitral valve and its apparatus. Of the 56 patients studied, 23 had completely normal study results, while in 33 (59%) results showed abnormalities of the mitral complex. The latter were divided into two groups: those having major mitral abnormalities (n = 12) and those with minor anomalies of the valve and apparatus (n = 21). The first group included patients with a supravalve stenosing ring, congenital mitral stenosis, mitral valve prolapse, and parachute mitral valve. Minor anomalies were classified as abnormalities of the papillary muscles, chordae tendineae, or combinations of both. No mitral malformations were found in a control group of patients. We conclude that two-dimensional echocardiography is a sensitive and accurate noninvasive method for assessing either significant or subtle forms of left ventricular inflow disease in patients with aortic coarctation. Its use is recommended for the acute medical and surgical management of these patients as well as for their long-term follow-up evaluation.

Recognition of residual post-operative shunts by contrast echocardiographic techniques☆

SUMMARYA bedside echocardiographic technique was used to detect and localize residual intracardiac shunts in 26 patients who had surgical repair of septal defects. Contrast echocardiography was performed through central venous and left atrial monitoring catheters at the same time as cardiogreen dye curves. Indicator dilution confirmed residual atrial defects in ten patients and ventricular defects in five. Contrast echocardiography indicated the presence and level of shunting in all 15 patients. Temporary flow through newly implanted septal patches was detected and differentiated from shunting across a true residual defect. The contrast echocardiographic technique using injections through the central venous and left atrial catheters as described detects and localizes right-to-left and left-to-right shunting. It is a safe and reliable method to evaluate residual intracardiac defects postoperatively.

Management Strategy for Very Mild Aortic Valve Stenosis

It is unclear how often patients with very mild aortic stenosis (gradients <25 mmHg) need interval follow-up. The purpose of this study was to define the determinants of disease severity progression and to propose appropriate management strategies. It is known that congenital aortic stenosis is a progressive disease that requires long-term follow-up at consistent intervals. We studied 89 patients with very mild aortic stenosis. Cox proportional hazard modeling was performed to ascertain predictors of morbidity and mortality. Events were defined as valve surgery or death. Of the original 89 patients, 7 died (92% survival); one death was sudden and unexplained and six were noncardiac. Eighteen individuals were lost to follow-up (10 not located and 8 refused participation). Twelve (17%) had valve surgery. The minimum time interval between initial diagnosis of very mild aortic stenosis and surgery was 4.6 years (mean, 14.0). Age at diagnosis, gender, initial gradient, initial gradient/age, and aortic regurgitation were found not to be predictive of outcome. However, the slope of the transaortic gradient [change of gradient/time (years)] was predictive of outcome (hazard ratio of 1.69; confidence interval, 1.4–2.2). At least 17% of these patients progress to require operation. For patients with a gradient slope <1.1, evaluation every 4 or 5 years is recommended. For patients with a gradient slope >1.2, evaluation every 1 or 2 years seems prudent.

Atrial Septal Aneurysm in a Patient With Autosomal Dominant Polycystic Kidney Disease

We describe a 6-year-old patient with autosomal dominant polycystic kidney disease, normal renal function, hypertension, and serial echocardiograms showing an aneurysm of the atrial septum. Intracardiac aneurysms have not previously been reported in patients with autosomal dominant polycystic kidney disease.

A rare form of isolated interrupted aortic arch: The value of two-dimensional echocardiography in the precatheterization evaluation

SummaryPreoperative evaluation of a 12-year-old girl, previously diagnosed as having a coarctation of the aorta, revealed a rare form of isolated interruption of the aortic arch. Two-dimensional echocardiography played an important role in the delineation of the defect and significantly reduced the need for invasive techniques. It helped to anticipate problems in reaching left-sided structures at cardiac catheterization, saving time and unnecessary trauma. This technique also ruled out associated intracardiac anomalies that were not detailed at cardiac catheterization.

Kawasaki Patients with Coronary Artery Calcifications Detected by Ultrafast CT Scan: A Population at Risk for Early Atherosclerosis

Background: Kawasaki disease produces a vasculitis which may injure the coronary arteries and predispose these patients to early atherosclerosis. Previously, case reports have documented that Kawasaki patients may develop coronary calcifications and may die of sudden death many years after their acute illness. Ultrafast computer tomography (UCT) has been successful at detecting coronary calcifications in adults with atherosclerosis. The goal of this study is to show that UCT can be utilized as a non-invasive technique to identify Kawasaki patients at high risk for coronary disease and who would require long-term surveillance through adulthood.Methods and Results: Eighteen patients ages five to twenty-one years old with a history of Kawasaki disease were enrolled. Each patient had an UCT of the heart. There were four patients with calcifications noted. All of the four patients had: a previous history of an aneurysm, were at least five years from their acute illness, and had the calcification correlate to the previous site of aneursym formation. A calcium score was determined by the Agaston method for each calcification. The Mayo clinic guidelines for adult atherosclerosis were then used to correlate these scores with the risk of future coronary artery disease. All four patients were at moderate or greater risk of future coronary disease.Conclusion: This study indicates that UCT can be used as an effective non-invasive methodology in Kawasaki patients to successfully identify the high-risk patients predisposed to developing potential early atherosclerotic coronary artery disease.