Beneficial effect of sodium dichloroacetate in muscle cytochrome C oxidase deficiency.
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Acidosis, Lactic↗
Biomedical subjects
O Milanesi
Publications and source records attributed to O Milanesi.
"Silent" patent ductus arteriosus and bronchopulmonary dysplasia in low-birthweight twins.
During a screening protocol of early echocardiographic diagnosis (ATL MK 600) and treatment of "silent" PDA in RDS preterms with BW < or = 1.750 kg, clinical data on premature twins were collected, including diagnosis of both PDA and BPD, to investigate whether twin birth influences PDA incidence and BPD development. Out of the 290 RDS preterms evaluated, 96 (33%) showed evidence of PDA, and a total of 79 (27%) developed BPD, 47 (16%) with associated PDA and 32 (11%) without PDA. Out of 238 singletons, 74 (31%) presented "silent" PDA and a total of 75 (31%) developed BPD, 44 (18%) with associated PDA, and 31 (13%) without PDA. In 52 other twins (18% of the total number of babies studied), 22 (42% of this subgroup) presented evidence of "silent" PDA, and 4 (8% of the subgroup), developed BPD, 3 with associated PDA (6% of the subgroup), and 1 without PDA (2% of the subgroup). From these data, it is inferred that that low-birthweight twins are at high risk for PDA hemodynamic complications during RDS, and may benefit from early induced ductal closure. Instead, in RDS twins, BPD was statistically less frequent (at the 99% C.L.) probably because twinning enhances fetal lung maturity, influencing enzymatic and nonenzymatic protective systems of lung defence.
Bronchopulmonary Dysplasia↗
Respiratory mechanics in infants and young children before and after repair of left-to-right shunts.
In an attempt to investigate the relationship between respiratory mechanics and pulmonary hemodynamics, we evaluated pulmonary function in 31 infants with left-to-right shunts and subsequent high pulmonary blood flow, undergoing cardiac surgery. Measurements were performed 1 d before and repeated 10 d and 4-5 wk after correction. The age of the patients ranged from 4 d to 24 mo, body weight from 2.7 to 11.8 kg. Pulmonary artery pressure, assessed by Doppler echocardiography, was preoperatively elevated in 23 patients (group 1), whereas it was within normal values in eight infants (group 2). Respiratory mechanics were measured using the single-breath occlusion technique in sedated infants. To evaluate specific compliance, functional residual capacity was determined by using an open circuit nitrogen washout technique. A reduced preoperative compliance value (mean with 95% confidence interval) was found in group 1: 34.8 (26.5-43.1) mL.kPa-1. After hemodynamic correction, a progressive significant (p < 0.01) improvement was demonstrated at 10 d and 1 mo with values of 47.5 (39.2-55.8) mL.kPa-1 and 56.5 (45.6-67.4) mL.kPa-1, respectively. A similar trend was noted evaluating specific compliance with values of 0.27 (0.24-0.30) kPa-1 and 0.44 (0.42-0.46) kPa-1, respectively before and after surgery. Preoperative functional residual capacity value was 130 (100-160) mL. In group 2, normal preoperative compliance values were obtained, without significant changes after surgery. In both groups, resistance was within the normal range both before and after surgical correction, and functional residual capacity did not change either. No correlations were found between compliance and pulmonary artery pressure and pulmonary blood flow values.(ABSTRACT TRUNCATED AT 250 WORDS)
Child, Preschool↗
Cardiac fibroma and heart transplantation.
Successful orthotopic heart transplantation was performed in a 38-day-old child with a fetal echocardiographic diagnosis of a left ventricular mass and in a 40-year-old woman with cardiac murmur since childhood and an echocardiographic diagnosis of asymmetric septal hypertrophy. Pathologic examination of the removed hearts, consisting of gross, histologic, immunohistochemical, and ultrastructural studies, led to the final diagnosis of cardiac fibroma. Both patients were alive and in good condition at 35 and 28 months, respectively, after operation.
Adult↗
Pulmonary atresia, intact ventricular septum, and Ebstein anomaly of the tricuspid valve. Anatomic and surgical considerations.
Pulmonary atresia with intact ventricular septum is a disorder that involves the whole right ventricle. An associated Ebstein deformity of the tricuspid valve is found in 10% of the cases, further complicating the anatomy and the function of the right ventricle. From January 1966 to December 1990, pulmonary atresia, intact ventricular septum, and Ebstein deformity of the tricuspid valve were observed in 11 cases in our institution; four of them were necropsy findings and the remaining seven were patients treated surgically. Of the latter, two were boys and five were girls (age range 1 day to 18 days). Two patients were managed by pulmonary valvotomy, three by systemic-pulmonary artery shunt, and two had a combination of the two. Operative mortality was 57%. Eight hearts were available for morphologic study. All had viscero-atrial situs solitus, D-loop ventricular structure, and normally related great arteries. A correlation between the degree of tricuspid valve dysplasia and right ventricular cavity size was observed in all. Furthermore, in five cases the anterior leaflet of the tricuspid valve obstructed the right ventricle at the ostium infundibuli level. In two of our surgical patients, a protruding anterior tricuspid valve leaflet was identified and excised and both patients survived. Ebstein anomaly of the tricuspid valve further complicates surgical management and outcome of pulmonary atresia and intact ventricular septum. Potential obstruction at the ostium infundibuli level should always be considered during repair. Various degrees of right ventricular inlet dysplasia, always present in this complex, may contraindicate an anatomic correction. In these cases a modified Fontan-type procedure should be considered as a valid surgical option.
Abnormalities, Multiple↗
Doppler and 2D echocardiographic diagnosis of congenital coronary artery fistulae to the right cardiac chambers: report of 3 cases.
We present three cases of coronary artery fistulae to the right cardiac chambers. The first was a 2-day-old neonate in congestive heart failure: 2D and Doppler echocardiography revealed a dilated proximal left coronary artery and a fistulous connection to the right atrium. The other two patients, respectively 4 and 3 years old, were asymptomatic and presented with a continuous heart murmur: a left coronary artery fistula into the right ventricle was detected by ultrasound in one, and a dilated proximal right coronary artery in the other. The diagnosis was confirmed in all three patients. The first patient was operated upon at 18 months of age; the second patient is awaiting surgery, and in the third patient the fistula was ligated at the age of 3 years. The possibility of ultrasound diagnosis without invasive procedures is suggested.
Child, Preschool↗
Electrical instability in patients undergoing surgery for atrioventricular septal defect.
Postoperative 24-hour Holter monitoring was performed in 106 patients with atrioventricular septal defect in order to identify the incidence of atrial and ventricular arrhythmias. Of the patients, 72 had separate atrioventricular orifices, including 13 with a small ventricular component to the defect, and 34 patients had a common atrioventricular orifice. Two groups of abnormal patients were found. First, patients with good electrical stability characterized by isolated atrial (9 patients) and ventricular (25 patients) extrasystoles falling in classes I and II of Lown. Second, patients with marked electrical instability characterized in one patient by repetitive atrial extrasystoles, in another by atrial flutter, in 2 by polymorphic ventricular extrasystole and in 8 by couplets or triplets. Electrical instability in individual patients was then compared, by means of logistic regression analysis, with operative, surgical and postoperative variables. There was no incidence of sudden death in our series. After surgical repair, ventricular arrhythmias were more frequent than atrial arrhythmias (33% vs. 10%) and were unrelated to the type of atrioventricular septal defect. Cardiac electrical instability after operation was significantly related to larger operative body size, higher postoperative end diastolic diameter of the right ventricle, larger size of the ventricular septal defect, coexistence of postoperative right bundle branch block and left anterior hemiblock. Conversely, the risk of arrhythmias was reduced by more recent operative data and by greater shortening fraction of the left ventricle.
Arrhythmias, Cardiac↗
[Congenital malformations of the mitral valve: clinical and morphological aspects].
The clinical, anatomic and functional features of 23 pts with congenital mitral valve malformation, seen at the University of Padua over a period of 10 years, are described. All pts underwent hemodynamic investigation and echo-Doppler evaluation. Diagnosis was confirmed at surgery in 17 cases, and at necropsy in six. In 9 pts mitral anomalies determined prevalent stenosis; the remaining 16 pts had moderate to severe mitral regurgitation. Associated cardiac malformations were present in 19 cases (82.6%). The clinical presentation occurred earlier in pts with mitral stenosis (16.9 months vs 6.55 years). The morphologic and echocardiographic features of each malformation are described. The term "valve dysplasia", as proposed by Becker et al to define the non-Ebstein malformations of the tricuspid valve, is herein applied to the congenital anomalies of the mitral valve in order to obtain a uniform and homogeneous approach to this vast spectrum of malformations.
Adolescent↗
Early screening and treatment of "silent" patent ductus arteriosus in prematures with RDS.
From January 1987 to December 1989, prematures with RDS weighing 1750 g or less admitted to the Neonatal Intensive Care Unite (NICU) were submitted from the third postnatal day to serial two-dimensional and pulsed Doppler (ATL MK 600) echocardiographic evaluation for "silent" patent ductus arteriosus (PDA). PDA was diagnosed in 36/175 prematures with RDS (20.5%). Thirty patients had indomethacin treatment and the PDA closed completely in 27 (90%); five needed a second course of indomethacin, that was effective in two (40%). Four RDS patients (4/36-11%) already weaned from the respirator, needed supplemental oxygen. The three non-responders and six other prematures with counterindications to the drug underwent surgical ligation (25%). As historical control, we retrospectively evaluated the population of preterm infants with RDS weighing less than or equal to 1750 g treated for hemodynamically significant PDA during three previous years before the screening protocol; in this group the echocardiographic and Doppler evaluations were done when congestive heart failure and pulmonary edema were clinically evident. In comparison, we found a reduced incidence of prematures with RDS treated for PDA, 7/120 (5.8%) a higher age at medical treatment (9 +/- 1.4 vs 4.4 - 2.3 days) and a larger prevalence of patients underwent ductal ligation (71.4%). These data show that early screening and treatment of "silent" PDA may result advantageous to improve the efficacy (90%) of indomethacin, in a critical time for the recovery of RDS, and furthermore decreasing the need of surgical ligation.
Birth Weight↗
Solitary aorta with bilateral ductal origin of non-confluent pulmonary arteries in pulmonary atresia with intact ventricular septum.
We report an exceptional case of pulmonary atresia with intact ventricular septum in which non-confluent pulmonary arteries were supplied by bilateral arterial ducts, the existence of which had been diagnosed in life. One should be aware of this arterial pattern, even when the ventricular septum is intact in the setting of pulmonary atresia, because it requires a different surgical approach than when atresia is associated with a ventricular septal defect, namely construction of bilateral shunts for palliation followed by reconstitution of the pulmonary arterial confluence for definitive repair.
Aorta↗
Does the respiratory distress syndrome in twins and singletons run different risks of persistent ductus arteriosus?
The incidence and evolution of patent ductus arteriosus (PDA) was evaluated in twins and preterm singletons with birth weight less than or equal to 1750 g admitted to our Department in 1987 for respiratory distress syndrome (RDS). Screening by echocardiography and Doppler-flow studies (AT MK 600) was performed on the third day of life. Out of 91 neonates who needed intubation and ventilation during this 12-month period (23.8% of admissions), 40 weighed less than 1750 g and of these 40, 14 were twins (35%). Hemodynamically significant PDA was documented in 13 patients; of these, only 5 were preterm singletons and 8 were twins. Two twins weighing less than 1000 g received no therapy for ductus closure; one ductus closed spontaneously, the other had an early demise. Three twins and 2 preterm singletons received indomethacin; one of the twins needed a second cycle for definitive ductus closure. Three twins and three preterm singletons underwent surgery, while one twin died on the 10th postoperative day. Screening and early therapy of PDA during RDS could be of great clinical importance. Twinning seems to play a role in the incidence and evolution of PDA and this needs to be evaluated in further studies.
Diseases in Twins↗
Repair of congenital malformations of the mitral valve in children: early and long-term results in 34 cases.
From March 1972 through December 1986, 34 children underwent surgical treatment of mitral valve dysplasia at our institution. There were 14 males and 20 females, with a mean age of 6 years (range, 5 months to 15 years). Eighteen patients had significant mitral valve insufficiency; 16 had significant mitral valve stenosis. Mitral valve reconstruction was possible in 30 patients (88.2%); replacement of the mitral valve with a mechanical prosthesis was necessary in the other 4 patients (11.8%), after at least 1 attempted reconstruction. Five patients died in the hospital (14.7%): 4 after conservative surgery, and 1 after mitral valve replacement. A 6th patient died 26 months after mitral valve replacement and resection of a subaortic membrane. Four patients required reoperation 2 to 66 months after conservative treatment: in 3 of these, the mitral valve was replaced; in the 4th, additional reconstruction was successful. Upon follow-up (range, 3 months to 14 years after surgery), 26 of the 28 surviving patients were asymptomatic; 2-dimensional and Doppler echocardiography performed in 22 patients after mitral valve repair showed normal pulmonary artery pressure in all, and moderate mitral valve dysfunction in 2. Our results indicate that reconstructive surgery for mitral valve dysplasia may prove effective and reliable in children, despite the frequent severity of valve malformation. We believe, therefore, that mitral valve repair should always be attempted in the pediatric population to avoid the disadvantages of prosthetic valve replacement.
Journal Article↗
Left ventricular size and function in double inlet left ventricle.
Using quantitative cineangiography we studied left ventricular size and function in 15 patients (aged 1 day to 15 years) with double inlet left ventricle. Our purpose was to assess how ventricular volume and performance change with age and palliative surgery in patients with and without restriction to pulmonary flow. Ventricular volumes were calculated using Simpson's rule method; end-diastolic volume was also stated as a percentage of predicted total (combined right and left) ventricular volume. Ejection fraction and systolic pressure to end-systolic volume ratio were calculated as indexes of ventricular function. Our results suggest that ventricular size is inadequate for a septation procedure in patients with restriction to pulmonary flow but increases after shunting operation. In patients without restriction to pulmonary flow, ventricular volume is adequate for a septation procedure during the first months of life, but it tends towards reduction, along with obstruction of the interventricular communication, after pulmonary artery banding. Ejection fraction is slightly lower than in left ventricle of heart with biventricular atrioventricular connexion, but it does not decrease with age, at least during the first 15 years of life. Systolic pressure to end-systolic volume ratio also does not decrease with age, but it is lower in patients with excessive pulmonary flow or atrioventricular valvar regurgitation.
Adolescent↗
Radionuclide evaluation of lung perfusion after the Fontan procedure.
Lung perfusion was evaluated in 19 patients in whom a Fontan operation had been performed at a mean age of 3.7 years. First pass and equilibrium data were acquired during the lung particle perfusion scan 0.5 to 7.9 years (mean 3.7 years) following the Fontan procedure. Abnormalities of lung perfusion were documented in 8 patients. Minimal underperfusion of small areas of either right or left lung were noted in 4 patients, while the remaining 4 had evidence of major perfusion defects, involving both lungs. The perfusion defects were localized, in the majority of cases, on the side where a palliative procedure had been performed before the Fontan operation: it is of note that all our patients without palliative procedures did not show abnormalities in lung perfusion. Major abnormalities of lung perfusion seemed related to possible intimal thrombosis or emboli due to prolonged polycythemia or to pulmonary vessel distortion due to long-standing shunts.
Child↗
Congestive heart failure secondary to cerebral arterio-venous fistula.
Arteriovenous fistula is a relatively rare cause of severe congestive heart failure in the newborn. An intracranial arteriovenous malformation associated with an aneurysm of the great vein of Galen is the most frequent of such malformations and, although well known, it remains a difficult diagnosis in the neonate because the clinical picture first presents with findings suggesting cardiac disease. The newborn with a vein of Galen aneurysm has volume and pressure overload on his myocardium; cyanosis is often present due to persistent fetal circulation; peripheral pulses are generally decreased in amplitude except in those arteries near the fistula where they are bounding, as an expression of a hyperdynamic status. A continuous murmur may be heard over the scalp, but this highly suggestive sign is often not present. The majority of newborns with intracranial arteriovenous fistula die very soon. Unfortunately, most of these infants are at first considered to have congenital heart disease and are, therefore, subjected to cardiac catheterization and angiography, with their well-known risks, before a correct diagnosis is made. Cross-sectional echocardiography, by demonstrating normal intracardiac anatomy, eliminates the need for an invasive investigation in a very sick neonate. Ultrasonography of the head will then provide a rapid and accurate demonstration of the aneurysm of vein of Galen. Cerebral angiography will complete the diagnosis in those cases in which a neurosurgical intervention is contemplated.
Heart Failure↗
The ventricular septal defect in complete transposition of the great arteries: pathologic anatomy in 57 cases with emphasis on subaortic, subpulmonary, and aortic arch obstruction.
Fifty-seven heart specimens with complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections) and ventricular septal defect were reviewed to establish the spectrum of morphology of the ventricular septal deficiency and to correlate the type of defect with presence of subarterial and aortic arch obstruction. The ventricular septal defect was single in 52 cases (27 perimembranous and 25 muscular) and multiple in five. A normal alignment between the outlet component and the rest of the muscular septum was present in 18 cases (10 perimembranous, five muscular, and three multiple). The defect was the consequence of septal malalignment in the other 39 specimens; 29 with rightward and 10 with leftward displacement of the outlet septum. Rightward displacement led to perimembranous defects in 16 cases and muscular defects in 12. In another instance, a perimembranous malalignment defect was associated with a muscular inlet defect. Subaortic stenosis due to either deviation of the outlet septum or prominence of the ventriculo-infundibular fold and septoparietal trabeculations was observed in 14 cases. Leftward displacement of the outlet septum was associated with one perimembranous and eight muscular defects and with multiple (muscular outlet plus muscular inlet) defects in another case. Of these, five cases showed subpulmonary stenosis. Aortic arch obstructions were present in 19 cases; 14 showed rightward malalignment of the outlet septum, which produced subaortic stenosis. These findings suggest two things: Unlike the situation in hearts with "normally related" great arteries, most defects in complete transposition result from malalignment of the outlet septum, with many being of the muscular type. Rightward or leftward displacement of the outlet septum frequently results in subaortic or subpulmonary stenosis, respectively. Aortic arch obstructions, although frequent, are not always associated with subaortic stenosis.
Adolescent↗
The risk of surgical treatment of tetralogy of Fallot: an appraisal.
The risk of surgical treatment of tetralogy of Fallot was analyzed with multivariate statistical techniques in a recent surgical series (1975-1983) of 159 corrected and 59 palliated patients. The overall mortality of correction was 12.6% and that of palliation was 15.3%. The significant and independent risk factors that increased the mortality of palliation were the use of a shunt other than the Blalock or its modifications (mostly Waterston) (P = 0.01), and very young age (P = 0.02). Risk factors that increased the mortality of correction were high left over right ventricular pressure ratio (P-RV/LV) (P = 0.02), persistent patency of the arterial duct (P = 0.02), other major associated lesions (P = 0.02), and the use of a transannular patch (P = 0.04). Cardioplegia significantly decreased the risk (P = 0.03). With present techniques, correction can be performed in favorable cases with a mortality approaching zero. Staged procedures neutralized the age related risk of correction, but Blalock shunts carried a mortality rate ranging from 9.5% at 1 month to 1.7% at 12 months of age. This was due to the lack of prostaglandins along with technical faults. Predicted two-stage mortality rates ranged in infants (less than 12 months) from 6.2 to 16% and compared favorably with our observed 27% mortality of primary correction. Our results show that the transannular patch becomes a fully expressed risk only in those patients with restrictive pulmonary arteries or isolated branch stenosis. This is because it decreases the force the right ventricle can generate in maintaining the cardiac output. The chances of survival, when compared with those of patients without transannular patch, are significantly less when the P-RV/LV is between 0.6 and 1.0. We therefore discuss the measures to decrease or neutralize this risk related to insertion of a transannular patch. Among the associated lesions, we discuss briefly the potential for residual right outflow tract stenosis, anomalous origin of the left anterior descending coronary artery and anomalous muscle bundle of the right ventricle.
Adolescent↗
Abnormal pulmonary artery branching in tetralogy of Fallot with "absent" pulmonary valve.
Microangiographic postmortem study in a case of tetralogy of Fallot with so-called absent pulmonary valve disclosed an abnormal branching pattern of the intrapulmonary arteries, but failed to detect any compression of intrapulmonary bronchi as a consequence of the distorted segmental arteries. The case also featured the absence of the ductus arteriosus, which should be considered a basic associated anomaly in the development of this malformative complex.
Humans↗