Determination of the optimal tolerance for MLC positioning in sliding window and VMAT techniques.

PURPOSE: Several authors have recommended a 2 mm tolerance for multileaf collimator (MLC) positioning in sliding window treatments. In volumetric modulated arc therapy (VMAT) treatments, however, the optimal tolerance for MLC positioning remains unknown. In this paper, the authors present the results of a multicenter study to determine the optimal tolerance for both techniques. METHODS: The procedure used is based on dynalog file analysis. The study was carried out using seven Varian linear accelerators from five different centers. Dynalogs were collected from over 100,000 clinical treatments and in-house software was used to compute the number of tolerance faults as a function of the user-defined tolerance. Thus, the optimal value for this tolerance, defined as the lowest achievable value, was investigated. RESULTS: Dynalog files accurately predict the number of tolerance faults as a function of the tolerance value, especially for low fault incidences. All MLCs behaved similarly and the Millennium120 and the HD120 models yielded comparable results. In sliding window techniques, the number of beams with an incidence of hold-offs >1% rapidly decreases for a tolerance of 1.5 mm. In VMAT techniques, the number of tolerance faults sharply drops for tolerances around 2 mm. For a tolerance of 2.5 mm, less than 0.1% of the VMAT arcs presented tolerance faults. CONCLUSIONS: Dynalog analysis provides a feasible method for investigating the optimal tolerance for MLC positioning in dynamic fields. In sliding window treatments, the tolerance of 2 mm was found to be adequate, although it can be reduced to 1.5 mm. In VMAT treatments, the typically used 5 mm tolerance is excessively high. Instead, a tolerance of 2.5 mm is recommended.

Servicio de Cuidados Intensivos Pediátricos del Hospital Vall d´Hebron: Diez años de soporte vital extracorpóreo / Pediatric Intensive Care Sevice of the Hosptial Vall d´hebron: Ten years of extracorporeal life support

La medicina intensiva se ha caracterizado desde sus inicios pro dar soporte no tanto a una patología específica sino al paciente que presenta fallo de la función de uno o varios órganos. Tradicionalmente, dicho enfoque ha consistido en optimiar la función de los órganos mediante el empleo de fármacos específicos (diuréticos, inotropos ... ) y de dispositivos mecániicos (ventilación mecánica). El cambio de paradigma de la última década consiste en pasar de apoyar al órgano enfermo a sustituir completamente su función, a la espera de la recuperación de la misma o bien de realización de un trasplante de ógano. Dicha sustitución de la función de órgano se inició en las Unidades de hemodiálisis, trasladándose después a las unidades de cuidados intensivos en forma de técnicas de depuración renal continua. Los desarrollos tecnológicos más recientes han permitido aplicar el mismo enfoque al fallo respiratorio y/o cardiaco refractarios mediante el empleo de la oxigenación con membrana extracorpórea y/o de los dispositivos de asistencia ventricular. La Unidad de Cuidados Intensivos Pediátricos del Hospital Vall d´Hebron inició su programa de Soporte Vital Extracorpóreo en el año 2002, habiendo superado en la actualidad el centenar de tratamietno con estas técnicas. La experiencia acumulada y los resultados actuales nos permiten afirmar que no encontramos en una nueva etapa en la que estas técnicas se han convertido en un cuidado estándar que ha transformado nuestra manera de afrontar la atención al niño críticamente enfermo (AU)

Intensive care medicine is characterized not by the care of a specific set of diseases but by treating those patients who present single or multiple organ failure. The trditional approach has been to optimize organ function by using specific drugs (diuretics, inotropes…) or mechanical devices (mechanical ventilation). During the last decade there has been a paradigmatic hange connsisting in substituting the function of the failing organ instead of trying to ameliorate it, until recovery ensues or an organ transplantation can be carried out. The concept of organ function substitution was introduced through haemodialysis in renal wardas and them brought to itnesive care units in the form of continuoud renal replacement therapy. The most recent technological improvements allow us to apply the same strategy to refractory cardiac and/or respiratory failure by means of extracorporeal membrane oxygentation and/or ventricular assist devices. The Extracorporeal Life Support Program at the Pediatric Intensive Care Unit of Vall d´hebron Hospital started in 2002, more than a hundred patints have been treated so far. The experience acquired and the msot recen outcomes attained allow us to state that these techniques have become a standard o care and that they have transformed the way we approach the care of the critically ill child (AU)

A method for accurate zero calibration of asymmetric jaws in single-isocenter half-beam techniques.

PURPOSE: To present a practical method for calibrating the zero position of asymmetric jaws that provides higher accuracy at the central axis and improves dose homogeneity in the abutting region of half-beams. METHODS: Junction doses were measured for each asymmetric jaw using the double-exposure technique and electronic portal imaging devices. The junction dose was determined as a function of jaw position. The shift in the zero jaw position (or in its corresponding potentiometer readout) required to correct for the measured junction dose could thus be obtained. The jaw calibration was then modified to introduce the calculated shift and therefore achieve an accurate zero position in order to provide a relative junction dose that was as close to zero as possible. RESULTS: All the asymmetric jaws from four medical linear accelerators were calibrated with the new calibration procedure. Measured relative junction doses at gantry 0° were reduced from a maximum of ±40% to a maximum of ±8% for all the jaws in the four considered accelerators. These results were valid for 6 MV and 18 MV photon beams and for any combination of asymmetric jaws set to zero. The calibration was stable over a long period of time; therefore, the need for recalibrating is seldom necessary. CONCLUSIONS: Accurate calibration of the zero position of the jaws is feasible in current medical linear accelerators. The proposed procedure is fast and it improves dose homogeneity at the junction of half-beams, thus, allowing a more accurate and safer use of these techniques.

NT-proBNP in cardiac surgery: a new tool for the management of our patients?

OBJECTIVES: Our aim was to determine NT-proBNP levels in patients undergoing cardiac surgery and if those levels are related to any of the baseline clinical characteristics of patients before surgery or any of the outcomes or events after surgery. METHODS: Prospective, analytic study including 83 consecutive patients undergoing cardiac surgery. Preoperatory and postoperatory data were collected. NT-proBNP levels were measured before surgery, the day of surgery, twice the following day and every 24 h until a total of nine determinations. Venous blood was obtained by direct venipuncture and collected into serum separator tubes. Samples were centrifuged within 20 min from sampling and stored for a maximum of 12 h at 2-8 degrees C before the separation of serum. Serum was stored frozen at -40 degrees C and thawed only once at the time of analysis. RESULTS: Mean age was 65+/-11.8 years. An Euroscore 6 was found in 30% of patients. NYHA classification was as follows: I:27.7%; II:47%; III:25.3%. Preoperative atrial fibrilation occurred in 20.5% of patients. After surgery 18.1% of patients required inotropes. Only one death was recorded. A great variability was found in preoperative NT-proBNP levels; 759.9 (S.D.:1371.1); CI 95%: 464.9 to 1054.9 pg/ml, with a wide range (6.39-8854). Median was 366.5 pg/ml. Preoperative NT-proBNP levels were unrelated to the type of surgery (CABG vs. others), sex, age and any of the cardiovascular risk factors. NT-proBNP levels were higher in high risk patients (Euroscore 6); (P=0.021), worse NYHA class (P=0.020) and patients with preoperative atrial fibrilation (m 1767 (2205) vs m 621 (1017); P=0.001). After surgery NT-proBNP levels started increasing the following day until the fourth day (P=0.03), decreasing afterwards (P=0.019). These levels were significantly higher in patients requiring inotropes after surgery (P<0.001). We did not find any relationship between NT-proBNP levels and complications rate (P=0.59). CONCLUSIONS: Preoperative NT-proBNP levels depend on preoperative patient status (Euroscore, NYHA class and cardiac rhythm) and they increase significantly after cardiac surgery. This increase is higher when postoperative inotropes are needed. We found no relation between NT-proBNP levels and complications rate. An association have been shown between NT-proBNP levels and the use of inotropes after cardiac surgery.

Association of Children with Heart Disease in the World: 10-year experience.

Thousands of children live in developing countries with untreated but correctable congenital heart disease (CHD), and most of them will die simple because they live in poor countries that do not have suitable medical/surgical facilities. The aim of the Association of Children with Heart Disease in the World is to support the operatory and teaching activities of a group of voluntary doctors engaged in the management of children with CHD in developing countries. Since 1992, the association has obtained 160 grants for foreign doctors who have been trained in our hospitals; performed 75 missions abroad, with more than 2150 cases evaluated and 404 cardiac operations performed; obtained millions of dollars worth of donated medical equipment; and constructed two cardiac surgery departments in Syria and Cameroon. Many efforts have been made, but much more can be done as we attain more experience and develop better coordination and collaboration between the international groups engaged in this emerging social need.

Glucemia capilar en ayunas como prueba de cribaje de la diabetes mellitus tipo 2 en la infancia

La descripción de fenotipos o de marcadores bioquímicos precoces de enfermedades metabólicas ha sido estudiada desde hace algunos años; varias de ellas, tienen su origen en la infancia o en la adolescencia, por lo cual, una intervención precoz podría evitar futuras complicaciones, mejorar la calidad de vida de estas personas y reducir los costos sanitarios. Objetivo: Describir un grupo de niños con alteraciones metabólicas que podrían desarrollar Diabetes Mellitus tipo 2 en la etapa adulta. Métodos: La glucosa capilar en ayunas (GCA) fue evaluada en 2.734 niños de Bailén (Andalucía, España). 51 niños fueron seleccionados en forma randomizada aunque 33 de ellos aceptaron continuar el estudio: 16 niños con GCA>5,55 mmol/l en dos ocasiones fueron considerados como Grupo A; el Grupo B estaba formado por 17 niños con GCA 7 en tres (3) que podrían ser considerados como pacientes diabéticos. La GPA (mmol/l) en el Grupo B fue 4,65 ñ 0,11. La circunferencia de cintura, la ratio proinsulina-insulina y los niveles de triglicéridos fueron significativamente diferentes entre los grupos. Conclusiones: La Diabetes Mellitus tipo 2 al igual que otras alteraciones metabólicas crónicas tendría un origen precoz, tal vez en la infancia o en la adolescencia. Por ello, un método simple y de fácil manejo como la determinación de la glucemia capilar en ayunas podría ser utilizado como prueba de cribaje de una población infantil aunque, a partir de estos datos sería necesario realizar cribajes similiares en otras poblaciones y comprobar la reproducción de los resultados (AU)

Phentolamine administration increases blood S100B protein levels in pediatric open-heart surgery patients.

AIM: Phentolamine administration during open-heart surgery shortens the cooling and rewarming phases of cardiopulmonary bypass (CPB) and hastens weaning from mechanical ventilation and extubation. Data on the effects of phentolamine on cerebral circulation and function in this setting are lacking. This study reports the cerebral effects of phentolamine using blood S100B protein levels and the middle cerebral artery pulsatility index (MCA PI). METHODS: Sixty pediatric patients undergoing congenital heart disease repair were randomly assigned to receive either phentolamine 0.2 mg kg(-1) i.v. (n = 30) or placebo (n = 30) before the cooling and rewarming phases of CPB. Samples for S100B measurement were collected at seven predetermined time-points before, during and after surgery. MCA PI values were recorded at the same times as sampling. RESULTS: S100B blood levels were higher in the phentolamine-treated group than in controls after rewarming (3.53 +/- 1.88 vs 1.58 +/- 0.53 microg l(-1); p < 0.001), remained persistently higher at the end of surgery (2.95 +/- 0.91 vs 0.79 +/- 0.21 microg l(-1); p < 0.001) and returned to normal ranges 12 h later than in the placebo group (p > 0.05). MCA PI values were also significantly higher at the end of surgery in the phentolamine-treated group (1.83 +/- 0.50 vs 1.22 +/- 0.34; p < 0.01). Cooling and rewarming times were shorter in the phentolamine-treated group (p < 0.01, for all). CONCLUSION: Despite improved peripheral vasodilatation and perfusion, phentolamine administration in pediatric open-heart surgery is correlated with increased cerebrovascular resistance and brain damage.

Capillary electrophoresis for measuring major and trace anions in thermal water and condensed-steam samples from hydrothermal springs and fumaroles.

A new application of capillary electrophoresis for measuring major and trace anions in thermal water and condensed-steam samples is presented. Ten fluid samples were collected from hydrothermal springs and fumaroles located in a volcanic zone of Deception Island, Antarctica. Anion separation was achieved in less than 6 min using indirect UV detection at 254 nm with a negative power supply (-15 kV). The electrolyte consisted of 4.7 mM sodium chromate, 4.0 mM electroosmotic flow modifier (OFM) hydroxide, 10 mM 2-(N-cyclohexylamino)ethanesulfonic acid and 0.1 mM calcium gluconate (pH 9.1). Major anions (Cl-, SO4(2), PO4H2+, and CO3H-) were measured using hydrostatic injection (10 cm for 30 s) at 25 degrees C. Trace amounts of anions (F-, Br-, and NO3-) were better determined by electromigration injection (4 kV, 10 s) at 15 degrees C. Good reproducibility of the migration times (<0.72% RSD), a satisfactory linear response and accuracy as well as acceptable detection limits were successfully obtained.

Shelhigh No-React porcine pulmonic valve conduit: a new alternative to the homograft.

BACKGROUND: The Shelhigh No-React pulmonic valve conduit is a new porcine conduit that is glutaraldehyde-treated and detoxified using a proprietary heparin process. In our institution it has been implanted in 25 patients. The aim of this present contribution is to evaluate the short-term follow-up after its implantation. METHODS: From November 1997 to August 1999, 25 patients (mean age, 20.2 years; range, 0.6 to 28.3 years) were operated on using this conduit. Seventeen patients underwent a Ross procedure for aortic valve disease, with the conduits implanted in anatomic position; 6 patients underwent right ventricular outflow tract reconstruction; 2 patients underwent the Rastelli operation. The follow-up was complete. Preoperative and postoperative two-dimensional echocardiography data were collected. RESULTS: There were two non-conduit-related deaths. Two conduits needed to be exchanged because of an increase in the gradient. Overall, all patients were improved in terms of New York Heart Association class. Comparison of preoperative and postoperative two-dimensional echocardiography gradient showed significant improvement. At the 30-month follow-up, no calcification was seen on the explanted conduits or on the two-dimensional echocardiography, although many of the patients are children. CONCLUSIONS: The Shelhigh conduits seem to be an alternative to homograft especially in infants. These experiences are preliminary, and longer follow-up is required.

Muertes violentas y consumo de alcohol / Violent deaths and alcohol consumption

Se realizó un estudio descriptivo, retrospectivo y longitudinal para conocer las características de los fallecidos por muertes violentas con cifras positivas de alcohol en sangre registrados por el Departamento Provincial de Medicina Legal de la provincia Guantánamo, en el periodo comprendido entre Enero de 1995 a Diciembre de 1998.Se utilizaron el libro de defunciones, el registro de archivos de protocolos de necropsias y el libro de registro de alcoholemia del Laboratorio Provincial de Toxicología para caracterizar la muestra escogida (n=226 casos). Se estudiaron las variables: porciento de alcoholemia, edad, sexo, ocupación y lugar de procedencia de los fallecidos, días, meses del año y áreas de ocurrencia, la etiología medicolegal, y las causas básicas y directas de las muertes.Se obtuvieron como resultados más relevantes que predominó la población joven entre las víctimas, los fines de semana y los meses de verano fueron los más frecuentes en la ocurrencia de los hechos violentos, y la etiología accidental, principalmente los de transito, la colisión y el shock traumático como las variables medico-legales más sobresalientes. (AU)

Fast-track congenital heart operations: a less invasive technique and early extubation.

BACKGROUND: Many novel techniques have been described for "minimally invasive" congenital cardiac operations to achieve an improved cosmetic result. There is little information on incorporation of such techniques into fast-track congenital heart operations. METHODS: We have developed an approach to fast-track congenital heart operations, which includes a cosmetic approach for repair of congenital heart defects without sacrificing adequate exposure or requiring specialized equipment, along with a simple approach to intraoperative anesthetic management that allows extubation in the operating room. The heart is exposed through a short midline skin incision and a full median sternotomy. The conventional technique of cannulation is performed. Between October 1997 and January 1999, 88 patients were operated on with this method. Cardiac anomalies included simple and complex ostium secundum atrial septal defect, sinus venous atrial septal defect, partial atrioventricular septal defect, simple and complex ventricular septal defect, and bicuspid aortic valve stenosis. RESULTS: There were no operative or late deaths. The majority of patients were extubated in the operating room or within 2 hours of operation. No patient underwent reoperation and the mean length of hospital stay was 3.9 days. Sternal instability or wound infection were not observed. CONCLUSIONS: We believe that our approach to fast-track congenital heart operation is safe and effective. The surgical technique provides good exposure and has excellent cosmetic results. Moreover, it is easy to learn and, if necessary, the surgeon can quickly gain direct access to the heart. The anesthetic management facilitates early tracheal extubation and a shorter duration of postoperative stay.

Revision of previous Fontan connections to total extracardiac cavopulmonary anastomosis: A multicenter experience.

BACKGROUND: Conversion to total extracardiac cavopulmonary anastomosis is an option for managing patients with dysfunction of a prior Fontan connection. METHODS: Thirty-one patients (19.9 +/- 8.8 years) underwent revision of a previous Fontan connection to total extracardiac cavopulmonary anastomosis at four institutions. Complications of the previous Fontan connection included atrial tachyarrhythmias (n = 20), progressive heart failure (n = 17), Fontan pathway obstruction (n = 10), effusions (n = 10), pulmonary venous obstruction by an enlarged right atrium (n = 6), protein-losing enteropathy (n = 3), right atrial thrombus (n = 2), subaortic stenosis (n = 1), atrioventricular valve regurgitation (n = 3), and Fontan baffle leak (n = 5). Conversion to an extracardiac cavopulmonary connection was performed with a nonvalved conduit from the inferior vena cava to the right pulmonary artery, with additional procedures as necessary. RESULTS: There have been 3 deaths. Two patients died in the perioperative period of heart failure and massive effusions. The third patient died suddenly 8 months after the operation. All surviving patients were in New York Heart Association class I (n = 20) or II (n = 7), except for 1 patient who underwent heart transplantation. Early postoperative arrhythmias occurred in 10 patients: 4 required pacemakers, and medical therapy was sufficient in 6. In 15 patients, pre-revision arrhythmias were improved. Effusions resolved in all but 1 of the patients in whom they were present before revision. The condition of 2 patients with protein-losing enteropathy improved within 30 days. CONCLUSIONS: Conversion of a failing Fontan connection to extracardiac cavopulmonary connection can be achieved with low morbidity and mortality. Optimally, revision should be undertaken early in symptomatic patients before irreversible ventricular failure ensues.

Intrapericardial teratoma in a newborn: a case report.

Intrapericardial teratomas are unusual tumors that often cause respiratory distress and might be lethal in the newborn. The purpose of this article is to present the clinical and pathological findings in a 12-day-old male successfully treated for a big intrapericardial teratoma. Given that the diameter of this tumor is generally related to the age at the time of diagnosis, the rarity of our case is the presence of a huge intrapericardial teratoma in a newborn. The surgical resection was lifesaving.

[The conversion of a modified Fontan procedure to a total extracardiac cavo-pulmonary conduit. The Medico-Surgical Cardiology Group]. / Conversione della procedura di Fontan modificata a connessione cavopolmonare totale extracardiaca. Raggrupamento di Cardiologia Medico Chirurgica.

BACKGROUND: After a modified Fontan procedure with atriopulmonary or atrioventricular conduit, some patients present stress intolerance, supraventricular arrhythmia, recurrent pleuropericardial or ascitic effusions, and protein-losing enteropathy, all of which are signs that the previous procedure has failed. The aim of this study was to evaluate the midterm outcome after surgical therapy for this condition. MATERIAL AND METHODS: Between August 1994 and December 1997, nine patients (6 males and 3 females), age 10 to 39 (mean 21.5) years, underwent conversion of previous modified Fontan procedure to total extracardiac cavo-pulmonary connection. Time from the previous procedure was 6 to 18 years (mean 10). Diagnosis was tricuspid atresia with pulmonary stenosis (n = 2), double-inlet left ventricle and concordant ventriculoarterial connection (n = 3), double-inlet left ventricle and discordant ventriculoarterial connection (n = 3), Holmes heart (n = 1). Nine patients presented decreased stress tolerance, seven had arrhythmia, five had pleuropericardial effusions and two had protein-losing enteropathy. In all but one patient, right atrial pressure was higher than 15 mmHg, while in six patients the cardiac index was less than 2 l/min/m2. A polytetrafluoroethylene non-valved conduit was interposed between the inferior vena cava and the right pulmonary artery for conversion in all patients. A bidirectional cavo-pulmonary anastomosis (modified Glenn) was associated in all patients. Evaluation was done by NYHA Class and by an arbitrary score assigned to patients based on 7 parameters. RESULTS: There was no perioperative mortality. All patients were clinically improved at a mean follow-up of 24 months (range: 3 to 46). No patient had effusions, and the arrhythmias disappeared in 4 patients and were controlled by medical therapy in one. The two patients with protein-losing enteropathy improved markedly within 30 days and the score dropped below 10 points. CONCLUSIONS: The conversion of the modified Fontan procedure to total extracardiac cavo-pulmonary connection improves clinical condition by decreasing the right atrium-pulmonary gradient and right atrial preload, and by providing a laminar cavo-pulmonary flow without any need for intracardiac anastomoses. This procedure should be undertaken early in this subset of patients, before ventricular failure ensues.

Serum ferritin as a component of the insulin resistance syndrome.

OBJECTIVE: In epidemiological studies, serum ferritin was the second-strongest determinant of blood glucose (after BMI) in regression models and the third-strongest determinant of serum insulin (after BMI and age). Its concentration also correlated positively with plasma triglycerides and apolipoprotein B concentrations, and negatively with HDL2 cholesterol. We hypothesized that serum ferritin could be a marker of insulin resistance. RESEARCH DESIGN AND METHODS: Oral glucose tolerance and insulin sensitivity (SI, minimal model method) were prospectively evaluated in 36 healthy subjects. The relationship between serum ferritin and metabolic control (as measured by HbA1c levels) was also studied in 76 consecutive NIDDM patients. RESULTS: In healthy subjects, log-transformed serum ferritin (LOGFER) correlated with basal serum glucose (r = 0.44, P = 0.007), but not with BMI, age, systolic or diastolic blood pressure, total cholesterol, VLDL cholesterol, HDL cholesterol, total triglycerides, VLDL triglycerides, serum insulin, or HbA1c (all P = NS). Identical results were obtained when the two lowest quartiles of serum ferritin were evaluated separately. However, in the two highest quartiles, LOGFER correlated with BMI (0.50, P = 0.02), diastolic blood pressure (r = 0.8, P < 0.0001), serum LDL cholesterol (r = 0.57, P = 0.01), VLDL cholesterol (r = 0.48, P = 0.03), total cholesterol and HDL2 and HDL3 subtractions of HDL cholesterol (r = -0.68, -0.76, -0.55, P = 0.001. < 0.0001, and 0.01, respectively), total triglycerides (r = 0.60, P = 0.006), HDL2/HDL3 quotient (P = -0.71, P = 0.001), VLDL triglycerides (r = 0.65, P = 0.004), and serum uric acid (r = 0.51, P = 0.03), but not with systolic blood pressure (r = 0.38, P = 0.15). After adjusting for BMI, only the correlations between LOGFER and diastolic blood pressure (r = 0.7, P = 0.002) and HDL2/HDL3 quotient (r = -0.63, P = 0.01) remained significant. Strong correlations between LOGFER and glucose area under the curve during oral glucose tolerance test (Pearson's r = 0.73, P = 0.001) and SI (r = -0.68, P = 0.001), which remained significant after controlling for BMI, were observed. LOGFER (beta = -0.44, P = 0.01) and BMI (beta = -0.52, P = 0.004) constituted independent predictors of insulin sensitivity in a multivariate analysis (R2 = 0.68). In 76 consecutive NIDDM outpatients, serum glucose (P < 0.00001) and LOGFER (P = 0.03) independently predicted the value of HbA1c (R2 = 0.40) in a multiple linear regression analysis. CONCLUSIONS: The correlations among serum ferritin and diastolic blood pressure, HDL quotient, glucose area under the curve, and SI suggest that serum ferritin could be a marker of the insulin resistance syndrome. Serum ferritin may also be an independent determinant of poor metabolic control in the diabetic patient.

Aortic pulmonary autograft implant: medium-term follow-up with a note on a new right ventricular pulmonary artery conduit.

BACKGROUND: The Ross operation has been applied to various aortic valve pathologies, particularly when somatic growth is an issue. However, associated cardiac disease and technical problems may limit its use with regard to associated procedures and issues of right ventricular outflow reconstruction. MATERIALS AND METHODS: From December 1992 to March 1998, 24 patients underwent aortic pulmonary autograft implantation. There were 14 males and 10 females, 15+/-10 years of age (mean +/- SD) (range 1 to 50 years), weighing 42.8+/-20 kg (mean +/- SD) (range 8 to 78 kg). Aortic insufficiency was present in 15 (62.5%) patients, stenosis in 8 (33.3%) patients, and valvar stenosis associated with left ventricular outflow tract obstruction in 1 (4.1%) patient. Etiology was rheumatic in 17 patients and congenital in 7. The Ross procedure was accompanied by a partial-Konno left ventricular outflow enlargement in one patient, and mitral valve annuloplasty, mitral commissurotomy, and tricuspid valve replacement in three other patients, respectively. The right ventricular outflow was reconstructed with a valved pulmonary homograft in 14 patients and with a Shelhigh No-React porcine pulmonary conduit in 10 patients. Evaluation was done by New York Heart Association (NYHA) Class and by echocardiography at a follow-up of 22.8+/-24 months (mean +/- SD) (range 3 to 63 months). RESULTS: There were no operative mortalities and no postoperative arrhythmias. One (4.1%) patient required intra-aortic balloon pump (IABP) support for 3 days, one (4.1%) patient died 2 years later of probable arrhythmia, and one (4.1%) patient required mechanical aortic valve replacement 2 years later for severe autograft insufficiency. Left ventricular ejection fraction was unchanged (preoperative 62.4%+/-30%, postoperative 64.2%+/-30% [mean +/- SD], [p = NS]) and no significant gradient was documented by echocardiographic Doppler in the right and left ventricular outflow tracts. The aortic insufficiency scale decreased from a mean of 3.9+/-0.2 to a mean of 1+/-0 (p < 0.01). NYHA Class decreased to I in all patients, from III (10) and II (14). CONCLUSIONS: The pulmonary autograft in the aortic position is suitable for aortic valve replacement in pediatric and adult patients with good medium-term results and in patients with rheumatic etiology, and it provides a desirable solution in the presence of associated pathologies, such as left ventricular tract obstruction or associated multivalvular disease. The development of new means of right ventricular outflow reconstruction must parallel the progress achieved for the left side.

[The role of a bidirectional cavopulmonary anastomosis in the correction and palliation of complex congenital cardiopathies]. / Ruolo dell'anastomosi cavo-polmonare bidirezionale nella correzione e palliazione delle cardiopatie congenite complesse.

Between May 1990 and January 1998, 68 patients underwent bidirectional cavo-pulmonary anastomosis. We evaluated all patients in whom the bidirectional cavo-pulmonary anastomosis was associated with additional pulmonary flow (group A) and those in whom it was associated with biventricular repair (group B). Group A included 23 patients (33.8%), 14 males and 9 females, mean age 25 years and 6 months (range 4 months-16 years). Diagnoses were double outlet right ventricle (6), univentricular heart (6), tricuspid atresia (5), congenitally-corrected transposition of the great arteries with ventricular septal defect and pulmonary stenosis (3), right isomerism (2) and pulmonary atresia with atrioventricular canal (1). Group B included 13 patients (19.1%), 6 males and 7 females, mean age 13 years and 7 months (range 1 year-37 years). Diagnoses were pulmonary atresia with intact ventricular septum (4), Ebstein's anomaly (3), tetralogy of Fallot (3), atrioventricular canal (1), hypoplastic right ventricle (1), and pulmonary and tricuspid insufficiency (1). Four patients (17.3%) in group A died in the postoperative period, whereas there was no postoperative mortality in group B. Follow-up data were available in 31 patients (19 from group A, 13 from group B). Mean follow-up was 1 year and 6 months (range 30 days to 6 years). Evaluation was done by NYHA class functional status. In group A, 14 patients are doing well (NYHA I or II), while five patients (26.3%) underwent Fontan operation with one death. All group B patients are currently doing well (NYHA class I or II). In group A, complications were pericardial effusion (7), transient superior vena cava syndrome (5), pleural effusion (4), chylothorax (1) and rhythm disturbance (1). Complications in group B involved neurological events (2), pleural effusion (1) and rhythm disturbance (1). Bidirectional cavo-pulmonary anastomosis can be associated with additional pulmonary flow with good short- and intermediate-term outcome. Concern remains for the ability to properly regulate the amount of effective pulmonary blood flow. Bidirectional cavo-pulmonary anastomosis can be associated with biventricular repair in patients with diminutive right ventricles, amenable to anatomic complete correction, with good clinical outcome.

Morphology of the atrioventricular valve in asplenia syndrome: A peculiar type of atrioventricular canal defect.

The high risk of atrioventricular (AV) valve regurgitation is a major point of concern in the natural history of patients with atrioventricular canal defect (AVCD) and asplenia syndrome (AS). The morphology of the common AV valve in patients with AVCD and AS was therefore studied and compared with the anatomy of patients with AVCD but with atrial situs solitus. We compared the anatomic features of hearts with common AV valve (ventricular loop; AV valve alignment on ventricles; number of leaflets; number and morphology of papillary muscles and relationship of the bridging leaflets with the ventricular septum) in the hearts of 33 patients with AVCD and asplenia syndrome (Group 1) with those of 44 patients with common AV valve, complete AVCD, and atrial situs solitus (Group 2). Hearts featuring asplenia syndrome showed a significantly higher occurrence of anomalies of the ventricular loop (p < .0001), right ventricular dominance (p < .01), and a reduced number of valvular leaflets and papillary muscles (p < .0005). On the contrary, hearts with situs solitus of the atria showed a significantly higher frequency of balanced ventricles (p < .0001) and a common AV valve with five leaflets and five papillary muscles (p < .000001). These data suggest that AVCDs in association with asplenia syndrome present morphologic peculiarities that may be considered as the substrate for the abnormal regurgitation. Recognizing the presence of these features could be of great value for the planning of a specific surgical treatment. These anatomic differences support the hypothesis of a genetic heterogeneity of AVCDs.

[Clinical, radiologic, ophthalmologic, and endocrine studies in the primary empty sella turcica syndrome (author's transl)]. / Estudio clínico-radiológico, oftalmológico y endocrino en la silla turca vacía primaria. A propósito de nueve casos.

The clinical, radiologic, ophthalmologic, and endocrine features of nine patients with the syndrome of primary empty sella turcica are described. Pneumoencephalography was diagnostic in the eight patients in whom it was performed, while the remaining case was diagnosed by computerized axial tomography. In two patients alterations of the visual fields were found, while another had a hypertensive eye fundus. Endocrine studies were normal in four patients; the following abnormalities were found in the remaining five cases: one case of partial deficiency of antidiuretic hormone associated to secondary amenorrhea, one case of functional galactorrhea, one case of lack of response of growth hormone to insulin hypoglycemia, one case of hypothalamic deficiency of the hypothalamus-pituitary-thyroid axis, and one case of panhypopituitarism. Pneumoencephalography gave the best diagnostic results but because of its dangers it must be performed only when computerized axial tomography gives negative or equivocal information and the patient is not a typical case (female sex, obese, multiparous, hypertensive, and or diabetid) in the forties.