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Biomedical subjects

D R Pieroni

Publications and source records attributed to D R Pieroni.

At least 19 recordsLinked to original sources

Neurodevelopmental outcome of infants with hypoplastic left heart syndrome.

The neurodevelopmental outcome of hypoplastic left heart syndrome in infants remains unclear. All 11 survivors of staged surgical repair of hypoplastic left heart syndrome received standardized neurodevelopmental assessments at one regional children's hospital. Seven children (64%) had major developmental disabilities. Quality-of-life outcomes must be considered when management options for children with hypoplastic left heart syndrome are evaluated.

Cerebral Palsy

Estimation of the ratio of pulmonary to systemic pressures by pulsed-wave Doppler echocardiography for assessment of pulmonary arterial pressures.

This study describes a method for estimation of the ratio of pulmonary to systemic pressures by pulsed-wave Doppler echocardiography. Sixty-eight patients ages 1 day to 68 years who underwent cardiac catheterization had Doppler studies of the right and left ventricular outflows. Preejection period (PEP), ejection time (ET) and mean acceleration to peak velocity (ACCm) were measured on each waveform. The expression: F = (PEP x ACCm)/ET was calculated for right and left ventricular outflows as an index of the effects that the interaction between ventricular contraction and afterload has on the shape of the Doppler waveforms generated in each outflow. The quotient of (F for the right outflow)/(F for the left outflow), or waveform contour ratio, was used to express the degree of pressure-dependent variability between each subject's right and left ventricular outflow tracings. The waveform contour ratio was strikingly similar to the ratio of systolic pulmonary to systemic pressures and also closely correlated to the ratio of mean pressures. The product of waveform contour ratio and arm systolic pressure gave a consistently accurate estimate of systolic pulmonary pressures. It is concluded that the present method can be used successfully for the noninvasive assessment of pulmonary arterial pressures.

Adolescent

Two-dimensional echocardiography in complete transposition of the great arteries.

Sixty three patients with complete transposition of the great arteries (d-TGA) were studied by two dimensional echocardiography in order to assess: great artery relationships; ventriculo-arterial connections; presence and nature of the left ventricular outflow obstruction (LVOTO). From the parasternal short axis view at the great arteries level, four different relationships were imaged: the aorta was anterior and to the right in 35 patients, the two vessels were in a direct anterior-posterior position in 15 or side by side in 3 and the aorta was anterior and to the left in 10. Differently from normals, early pulmonary bifurcation of the artery arising from the left ventricle was imaged in 42 patients (84%) from the apical approach and in 36 (72%) from the subcostal longitudinal view. Left ventricular outflow tract obstruction was diagnosed in 29 patients (46%) and two distinct types of obstruction were differentiated: fixed and dynamic. We conclude that information derived from two-dimensional echocardiography is a valuable tool for the medical management, cardiac catheterization and surgical treatment of infants and children with d-TGA.

Adolescent

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.

Aneurysm, Infected

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.

Adolescent

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