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Biomedical subjects

D R Pieroni

Publications and source records attributed to D R Pieroni.

At least 37 records · Page 2Linked to original sources

Aortoplasty and aortic valve replacement for annuloaortic ectasia in an infant with Marfan's syndrome, forme fruste.

This report describes our experience with a 4-month-old infant with annuloaortic ectasia in whom intractable heart failure developed because of acute exacerbation of aortic regurgitation. He underwent an aortoplasty with aortic valve replacement and made an uneventful recovery. Since there has been no report of a successful repair of this condition in infancy, the case is presented and relevant problems are discussed.

Aorta↗

Two-dimensional echocardiographic recognition of the right aortic arch.

A mirror-image right aortic arch (RAA) is frequently associated with congenital heart disease. The chest roentgenogram with or without contrast remains the noninvasive diagnostic method of choice. While 2-dimensional (2-D) echocardiography has been used to elucidate the left aortic arch (LAA), detailed description of the technique for RAA has not been reported. This study was pursued to delineate the 2-D echocardiographic approach to the patient with RAA. Twenty-seven patients in this study had LAA (Group A) and 27 had RAA (Group B). The 2-D echocardiographic examinations concentrated on the standard suprasternal long-axis (SSNL), parasternal long and short axes, and the subcostal abdominal short-axis views. When the SSNL failed to demonstrate LAA, an alternate position for RAA was utilized. The SSNL correctly identified the LAA in all Group A patients, but in Group B it located only the ascending aorta. This simulated an interrupted aortic arch anomaly. Transducer realignment to position 2 confirmed RAA in all Group B patients. In the parasternal axes, the left descending aorta was detected posterior to the atrioventricular groove or the left ventricle in every Group A patient but in no Group B patient. The descending aorta was found to the left of the spine on the abdominal short-axis view in both groups. The 2-D echocardiographic technique proposed for RAA is simple, rapid, and definitive. It should be pursued whenever LAA cannot be demonstrated, especially in children suspected of having a congenital heart defect. Anticipation of RAA can expedite chest and cardiovascular surgery.

Adolescent↗

Atrial right-to-left shunt in infants with respiratory and cardiac distress but without congenital heart disease. Demonstration by contrast echocardiography.

Contrast echocardiography was used to detect atrial right-to-left shunts in 20 term infants; there were 10 cyanosed infants who had severe respiratory distress and signs of cardiac failure but no apparent congenital heart disease and 10 noncyanosed infants with no respiratory distress and no signs of cardiac failure who underwent exchange transfusion for hyperbilirubinemia. All of the venous contrast echocardiograms in the cyanosed infants showed an atrial right-to-left shunt with a dense contrast effect in the left atrium and aorta but little in the right ventricle. A sparse effect in the left atrium and a dense one in the right ventricle were recorded in infants with resolving respiratory distress and in three infants from the control group. The other seven infants in the control group showed no right-to-left shunt. Conventional M-mode echocardiograms showed no evidence of congenital heart disease in any of the 20 infants. This contrast echocardiographic technique was useful for demonstrating an atrial right-to-left shunt in cyanosed term infants with neonatal respiratory problems and signs of congestive failure but no apparent congenital heart disease.

Cyanosis↗

Two-dimensional echocardiographic recognition and surgical management of aortopulmonary septal defect in the premature infant.

An aorticopulmonary septal defect (APSD) results from failure of proper conotruncal separation. The hemodynamic consequences of this lesion closely resemble that of other large left-to-right shunt defects, especially a patent ductus arteriosus (PDA). Failure to differentiate these 2 abnormalities has not infrequently led to an inappropriate surgical approach in pursuit of the far more common PDA. This report describes the two-dimensional echocardiographic (2-DE) recognition of an APSD in 2 premature infants. Survival of these delicate neonates indicates that successful surgery can be performed even in small infants. A thorough 2-DE examination for an APSD is recommended for any infant before proceeding to surgery for a suspected PDA, especially when cardiac catheterization has not been performed.

Aorta↗

Residual shunting in the early postoperative period after closure of atrial septal defect: echocardiographic comparison of patch materials.

Residual shunting after surgical closure of septal defects is a common postoperative complication. In this study, contrast echocardiography was used to assess the effect of different surgical patch materials on early postoperative residual shunting. The study consisted of 44 patients (aged 3 days to 64 years) with simple or complex atrial septal defects. Total pulmonary-to-systemic flow ratios ranged form 1.8:1 to 4.0:1. Three methods were used to close the atrial septal defects: primary suture closure (n = 7), patching with thin, knit Teflon fabric (n = 13), and patching with thicker, low porosity, knit Teflon fabric (n = 24). Contrast echocardiographic injections were performed through central venous and left atrial lines positioned at operation for monitoring purposes. Ten of the 44 patients had residual shunts. In five of them, daily contrast studies showed progressive diminution in shunting with eventual resolution, but in the other five patients, shunting persisted beyond the first 5 postoperative days. Three of the latter five required reoperation for actual residual anatomic defects. No patient whose atrial septal defect was closed by either direct suture or thick, low porosity Teflon fabric had shunting detected at any time postoperatively. Our data confirmed temporary leakage across newly implanted intracardiac patches. However, shunts that persist beyond the first postoperative week indicate true anatomic residua and not porous patch material. If a patient's recovery is complicated, use of contrast echocardiography can determine whether a residual shunt is a causative factor. In patients with complex lesions in whom continued shunting during the early postoperative period may cause serious hemodynamic consequences, heavier, low porosity patch material can be useful.

Adolescent↗

Closed transventricular pulmonary valvotomy in infants.

In an effort to reassess the efficacy of closed transventricular valvotomy in infants with severe pulmonary stenosis, we reviewed 24 consecutive patients who underwent closed transventricular valvotomy. The age range was 1 day to 11 months (median 53 days), with 10 patients under 1 month and 21 under 6 months of age. The weight range was 2.6 to 9.4 kg (median 4.1 kg). The long-term results were assessed by comparing the postoperative to the preoperative clinical and hemodynamic data. The 20 survivors were followed up for 3 to 133 months (median 54 months). All were asymptomatic upon the last follow-up visit, and their electrocardiograms and chest x-ray films were normal or improved. In 12 patients who had cardiac catheterization 7 to 85 months (median 50 months) after operation, the range for the right ventricular-to-left ventricular, or systemic arterial, peak systolic pressure ratio (RV:LV) was 0.97 to 1.7 preoperatively (mean 1.31) and 0.22 to 0.94 postoperatively (mean 0.42) (p less than 0.001). In order to assess the significance of the RV size for the surgical survival, we measured the preoperative RV end-diastolic volume (RVEDV) in 17 patients. Twelve patients had a normal or enlarged RV and all survived the operation, whereas two of the five patients with an RVEDV more than 2 SD below the normal mean (RVEDV less than 23 ml/m2) died postoperatively (p = 0.075). We conclude that closed transventricular valvotomy can be done successfully in infants with severe pulmonary stenosis and an RV which is not small. The risk of cardiopulmonary bypass is avoided and good long-term results can be obtained. We also present evidence that a small RV (RVEDV less than 23 ml/m2) is a potentially important predictor of the surgical risk.

Blood Pressure↗

Rapid elimination of quinidine in pediatric patients.

The oral absorption and elimination of quinidine in pediatric patients was studied. Single oral doses of quinidine sulfate were administered to 13 patients ranging in age from 4 to 22 years of age. Serum quinidine concentration reached a peak within 30 minutes to two hours after drug administration. The serum half-life of quinidine varied from 2.5 to 6.7 hours and was, on the average, shorter than the reported estimates for adult volunteers and cardiac patients (means ranging from 4.9 to 7.3 hours). Hence more frequent dosing or the use of slow-release preparations may be necessary in some pediatric patients in order to avoid excessive fluctuation in serum drug concentrations over a dosage interval. The oral dose clearance of quinidine (ie, oral dose divided by the area under the serum concentration time curve) varied over a threefold range, from 0.151 to 0.570 liter/hr/kg, and was found to correlate inversely with age (r = .80). In comparison with mean clearance estimates that have been reported for normal adult volunteers (0.293 +/- 0.078 liter/hr/kg), children less than 12 years of age (0.461 +/- 0.117 liter/hr/kg) were found to have significantly higher clearances, whereas no difference was observed between older children (0.287 +/- 0.101 liter/hr/kg) and adults. Inasmuch as the average steady-state serum drug concentration for a given daily maintenance dose is directly related to clearance rate, children less than 12 years of age may require a higher dosage of quinidine on a per kilogram of body weight basis. Proper selection of quinidine dosage, careful adjustment of dosage according to age, and regular monitoring of drug response and serum drug concentration are essential steps to a rational management of quinidine therapy in children.

Administration, Oral↗

Two-dimensional echocardiographic recognition of coronary artery disease in Kawasaki disease. A case report.

Two-dimensional echocardiography (2-DE) was used to demonstrate bilateral coronary artery disease in a 2-month-old child presenting with a mucocutaneous lymph node disease. The classic clinical and laboratory signs of this illness, now known as Kawasaki disease, were reviewed. Identification of the high risk patient prone to developing pericarditis and coronary perivasculitis with subsequent congestive heart failure or myocardial damage is essential for lowering morbidity and mortality rate. The 2-DE successfully demonstrated a large right coronary aneurysm and a markedly abnormal left coronary artery. The 2-DE should be used in every patient suspected of having Kawasaki disease not only to determine coronary artery involvement but also to guide medical management.

Coronary Disease↗

Familial pulmonary atresia. Its occurrence with a ventricular septal defect.

To our knowledge, this is the first reported case of pulmonary atresia with a ventricular septal defect that occurred in a father and his son. A multifactorial mode of determination that resulted from an interaction between genetic predisposition and environmental factors was assumed, since the family history did not show other members with congenital heart defects. Based on the background and on information from the multifactorial theory of inheritance, a counseling plan was formulated for these parents. A similar approach is recommended for the many surgical survivors of previously uncorrectable complex congenital heart defects as they approach reproductive age.

Abnormalities, Multiple↗

The sensitivity of contrast echocardiography in detecting intracardiac shunts.

While contrast echocardiography has been shown to delineate intracardiac shunts, no information is available concerning the sensitivity of the method. This study establishes the relative sensitivity of the technique in detecting and localizing shunts by comparing the contrast echocardiograms with the simultaneously recorded cardiogreen dye curves in 26 children during cardiac catheterization. The echocardiograms substantiated isolated intracardiac shunting as small as 5%. There were no false-positive or false-negative echocardiograms in patients with or without shunt defects. This report demonstrates the contrast echocardiographic technique to be extremely sensitive in detecting intracardiac communications. It reviews the diagnostic patterns necessary for diagnosis and proposes the technique as a sensitive, safe, and accurate alternate method of study in the cardiac catheterization laboratory.

Adolescent↗

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.

Adolescent↗

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

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.

Adolescent↗

The superior QRS axis in ostium primum ASD: a proposed mechanism.

The influence of abnormal hemodynamics, ventricular hypertrophy, and right bundle branch block on the AQRS was studied pre- and post-operatively in 29 patients with OPSD. The AQRS markedly diminishes with the surgical correction of abnormal hemodynamics and the subsequent resolution of RVH or BVH. With the persistence of ventricular hypertrophy postoperatively or the surgical induction of RBBB, the AQRS either remains unchanged or, in the latter instance, becomes more superior and rightward. The dependence of the superior AQRS on these factors suggests that a left anterior hemiblock is not responsible for this AQRS. In OPSD early activation of the posterobasal region of the left ventricle through an abnormally short posterior fascicle results in a minimal superior AQRS which is then exaggerated in the presence of abnormal hemodynamics, ventricular hypertrophy, or RBBB. Thus, the superior AQRS in OPSD with associated RBBB does not represent a true bifascicular block and has a different natural history and clinical significance.

Adolescent↗

Echocardiography in atrioventricular canal defect: a clinical spectrum.

Echocardiographic examinations were performed in 30 children with atrioventricular (A-V) canal defect to determine the diagnostic value of recording A-V valve motion. Although a wide spectrum of abnormal movement was observed, four main diagnostic types emerged. Type 1, prolonged mitral-septal approximation during diastole, resulted from anterior displacement of the cleft anterior mitral leaflet. Type 2, the most diagnostic motion, was recorded when the common anterior A-V leaflet traversed the interventricular septum. Type 3 motion reflected posterior displacement of the septal tricuspid leaflet, which appeared to arise from the interventricular septum. Normal posterior diastolic motion of the septal leaflet was never recorded. Type 4, double contour of the mitral valve during systole and marked duplication of the systolic image, corresponded to irregularities in the mitral valve. When these patterns are recorded in a patient with congenital heart disease the diagnosis of A-V canal defect can be made with confidence.

Adolescent↗