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Biomedical subjects

C Marcelletti

Publications and source records attributed to C Marcelletti.

At least 19 recordsLinked to original sources

Neonatal pulmonary autograft implantation for cardiac tumor involving aortic valve.

We diagnosed in a 4-day-old neonate a cardiac tumor involving the left atrium, left atrioventricular junction, left ventricular outflow tract, and aortic valve with severe subvalvular and valvular aortic stenosis. The critical involvement of the aortic valve and the scarcity of neonatal cardiac donors led us to perform a successful replacement of the aortic root with a pulmonary autograft, using a very small homograft for the native pulmonary valve (Ross operation).

Aortic Valve

Risks of acute renal failure after cardiopulmonary bypass surgery in children: a retrospective 10-year case-control study.

To our knowledge there are no case-control studies that have examined the main risk factors for acute renal failure (ARF) following cardiopulmonary bypass surgery in children. We therefore evaluated the potential risk factors in a large retrospective case-control study. Sixty-one of 2262 children (2.7%) developed postcardiopulmonary bypass surgery ARF requiring peritoneal dialysis (PD) from 1982 to 1991. Fifty-eight of 61 cases (median age 8.5 months) were selected by systematic sampling and matched with 176 controls who did not develop ARF. The four matching variables were age, cardiopulmonary bypass and circulatory arrest duration, and year of operation. Mortality rate was 79% in cases (controls: 18%). Forty-three of 48 of the deceased cases did not recover renal function: no renal cause of death was found; 13 of 61 cases survived and recovered renal function. Multiple regression analysis showed the following significant risk factors for postcardiopulmonary bypass surgery ARF: central venous hypertension > 12 h (odds ratio (OR) 9.6); systolic arterial hypotension > 12 h (OR 8.9); dopamine dosage > 15 micrograms/kg/min (OR 3.0); adrenaline (OR 5.9) and isoproterenol (OR 13.5) use. High preoperative serum creatinine, cyanosis, and vasodilator use were not significant risk factors.(ABSTRACT TRUNCATED AT 250 WORDS)

Acute Kidney Injury

Echocardiographically guided repair of tetralogy of Fallot.

Although 2-dimensional, Doppler, color flow echocardiography is accepted as a safe diagnostic method to guide the surgical treatment of certain heart defects, cardiac catheterization remains mandatory for patients with tetralogy of Fallot. Based on the excellent diagnostic correlation between echocardiography and angiocardiography, a policy of echo-guided primary repair of uncomplicated, selected cases of tetralogy of Fallot was introduced at Ospedale Bambino Gesù. In the last 5 years, of 139 patients who had surgery for tetralogy of Fallot, 105 underwent primary repair. In 56 patients (53.3%), surgery was guided only by echocardiography (group I). In the remaining 49 patients (46.7%) (group II), unclear imaging of cardiovascular anatomy (n = 23), or echo-suspected associated malformations (n = 26) prompted cardiac catheterization. The 2 groups did not differ in age and weight at surgery. Intraoperative examination did not show diagnostic errors in patients of group I; cardiac catheterization and surgery confirmed the suspected associated anomalies in 16 of 26 patients of group II (38.4% false-positive). Echocardiography showed an overall sensitivity of 100% and specificity of 85% for detection of associated malformations. In conclusion, echocardiography proved to be adequate for selection of patients with uncomplicated forms of tetralogy of Fallot for primary repair.

Cardiac Catheterization

[Cardiac retransplantation: is it justified in childhood?].

A 7-year-old boy had undergone heart transplantation (HT) at 1 year of age. The immunosuppressive regimen consisted of cyclosporine and azathioprine (Cy+Aza). During the follow-up there were 7 episodes of moderate rejection: 4 of them occurred during the first 3 months. He had cytomegalovirus (CMV) seroconversion 4 years after HT. After 5 years since he underwent primary HT, cardiac catheterization and selective coronary angiography, performed on a yearly basis, showed triple vessel occlusive disease. The treadmill test was positive. During the following year, the patient's clinical condition deteriorated: in May '92 he underwent retransplantation. Cross-match was negative and there were no common HLA-DR antigens between the first and the second donor heart. Only 1 rejection episode occurred during the first 18 months of follow-up. Despite the shortage of donor hearts we feel that retransplantation is justified as an elective procedure in pediatric patients with cardiac allograft vasculopathy (CAV).

Biopsy

[Central cyanosis in a newborn infant due to a prominence of the eustachian valve].

A 3-day-old neonate with severe central cyanosis was found to have a right to left shunt across a secundum atrial septal defect, due to prominence of the Eustachian valve. Two-dimensional echocardiography with Doppler and color Doppler allowed a precise morphological and pathophysiological diagnosis. Cardiac catheterization and angiocardiography confirmed the diagnosis. The patient is asymptomatic 12 months after surgical excision of the Eustachian valve and closure of the atrial septal defect, performed at two months of age.

Angiocardiography

Total cavopulmonary direct anastomosis: a logical approach in selected patients.

A 2.5-year-old boy with a diagnosis of situs solitus, tricuspid atresia, anatomically corrected malposition of the great arteries (S,D,L), left juxtaposition of atrial appendages, and pulmonary stenosis underwent successful total cava-to-pulmonary connection by means of a superior vena cava-to-pulmonary artery end-to-side anastomosis associated with an inferior vena cava-to-pulmonary artery direct anastomosis. Anatomic features and surgical technique are described.

Abnormalities, Multiple

[The bidirectional cavo-pulmonary anastomosis: a clinico-functional assessment in a medium-term follow-up].

BACKGROUND: The bidirectional cavo-pulmonary anastomosis is a useful surgical procedure for patients with complex congenital heart disease characterized by univentricular physiology. This operation significantly increases effective pulmonary blood flow and rarely causes distortion of the main pulmonary arterial branches. The aim of this study was to evaluate the clinical and pathophysiologic consequences of bidirectional cavo-pulmonary anastomosis as well as the effectiveness of this surgical approach as a definitive palliation. METHODS: Clinical, hemodynamic and functional data of 12 patients who had undergone a bidirectional cavo-pulmonary anastomosis were reviewed. Each patient underwent clinical examination, chest x-ray, ambulatory EKG, bidimensional color-Doppler echocardiography, spirometric analysis, lung perfusion scintigraphy, cardiac catheterization and angiography, and stress test. Stress test data were compared to those obtained by a normal population of age-matched children. RESULTS: After a mean follow-up interval of 28 +/- 20 months all patients showed a good clinical condition. Improved oxygenation, exercise tolerance and growth velocity were noted in all patients. Only 34% of patients showed abnormalities of chest x-ray, 29% manifested significant arrhythmias during ambulatory EKG and 20% showed abnormal ventricular function echocardiographically. Spirometry was normal in all patients, while lung perfusion scintigraphy was constantly abnormal with right/left and upper/lower perfusion mismatch. At cardiac catheterization a reduction of end diastolic volume (from 106.9 +/- 50.6 to 67.3 +/- 41.6 ml, p < 0.05) and end systolic volume (from 58.0 +/- 27.9 to 32.5 +/- 33.5 ml, p < 0.05) was noted. The functional evaluation of these patients was highly abnormal due to an impaired response to effort as evidenced by work time (p < 0.0001 vs normal) and stress-induced cardiovascular modifications. CONCLUSIONS: On the basis of these findings, we suggest that: 1) bidirectional cavo-pulmonary anastomosis is useful as an intermediate step towards a Fontan procedure, as it improves the hemodynamic performance of the systemic ventricle; 2) conversely, bidirectional cavo-pulmonary anastomosis should not be considered a form of definitive palliation for complex congenital heart disease because in these patients the response to exercise remains unsatisfactory.

Adolescent

Early identification of patients at risk for sinus node dysfunction after Mustard operation.

We studied 60 patients who had survived the Mustard procedure for transposition of the great arteries, performed between the ages of 2 days and 24 months (mean 4.51 +/- 3.79). All patients were given a postoperative 24-hour dynamic electrocardiogram at 15 days, 1 year and 3 years and then every 2 years. Those who were found to have sinus node dysfunction during follow-up, were given a dynamic electrocardiogram every 3-6 months. The average follow-up period was 38.7 +/- 19.8 months, median 36. Sinus node dysfunction was detected during follow-up in 20 patients (33.3% of the total), 8 of whom had had a pacemaker inserted. Risk factors for late development of sinus node dysfunction were found to include prolonged cross-clamping of the aorta during surgery (P = 0.003), especially over 50 minutes (relative risk 3.5:1), and the presence of even transient sinus node dysfunction after surgery (P = 0.006). These observations suggest, first, that sinus node dysfunction may develop after the Mustard operation as a combined effect of extensive atrial surgery and a long period of myocardial ischemia and, second, that the presence of the disease immediately after the operation sometimes indicates that it will recur or persist during follow-up.

Arrhythmias, Cardiac

"Acquired" subvalvular aortic stenosis after repair of a ventricular septal defect.

Of 353 children who underwent surgical repair of a congenital heart defect, including closure of a ventricular septal defect (VSD), 12 patients (four with tetralogy of Fallot, five with a VSD, and three with a double-outlet right ventricle) developed subaortic stenosis, which was diagnosed one to six years after the surgical procedure. Five patients required surgical treatment of the subaortic stenosis, and one required percutaneous balloon angioplasty. Postsurgical subaortic stenosis appears to be an uncommon progressive acquired disease.

Aortic Stenosis, Subvalvular

Early congestive heart failure due to origin of the right coronary artery from the pulmonary artery.

We describe a two-month-old infant with early congestive heart failure due to anomalous origin of the right coronary artery from the pulmonary artery. The diagnosis was made by two-dimensional and color flow Doppler echocardiography, confirmed by angiocardiography, and the case was successfully corrected at surgery. As opposed to the more frequent anomalous origin of the left coronary artery from the pulmonary trunk, this anomaly generally does not cause any typical clinical finding, often becoming an autoptic or surgical surprise after infancy or in adult age.

Coronary Vessel Anomalies

Ductus arteriosus in pulmonary atresia with and without ventricular septal defect. Anatomic and functional differences.

The pulmonary circulation is dependent on the ductus arteriosus in all patients with pulmonary atresia and intact ventricular septum and in some with pulmonary atresia and ventricular septal defect (tetralogy of Fallot type). To assess the time of ductal closure in these two patient categories, we compared the ages at first operation in 58 patients with pulmonary atresia and intact ventricular septum and 32 with pulmonary atresia and septal defect. The age distribution differed significantly between the groups. Whereas 90% of the children with intact ventricular septum required surgery in the first week of life, 50% of those with ventricular septal defect underwent surgery after the first month and 25% after the third month. The previously described and now confirmed anatomic differences of ductus arteriosus or different levels of endogenous prostaglandins may explain persistent patency of the ductus in pulmonary atresia with ventricular septal defect. The phenomenon may have important clinical implications regarding the timing and choice of surgical procedure.

Abnormalities, Multiple

Bidirectional cavopulmonary anastomosis in congenital heart disease. Functional and clinical outcome.

The bidirectional cavopulmonary anastomosis is a surgical procedure suitable for patients with cyanotic congenital heart disease and univentricular physiology. This operation is able to increase the effective pulmonary blood flow without any additional load on the cardiac work and without any further distortion on the pulmonary artery branches. The cavopulmonary anastomosis can represent the first stage for patients destined for Fontan repair or a definitive palliative operation in high risk Fontan candidates. In order to test the hypothesis of a definitive palliation by cavopulmonary anastomosis in this kind of patients, we evaluated the hemodynamic data before and after this surgical approach and compared these data with their clinical and functional outcome. We evaluated 74 patients submitted to bidirectional cavopulmonary anastomosis by either hemodynamic or functional evaluation. End-diastolic and end-systolic ventricular volumes were significantly reduced by bidirectional cavopulmonary anastomosis (p less than 0.0005). Despite these data and a normal ambulatory ECG, spirometry and echocardiographic analysis, the stress test showed discouraging results. In fact, mean work time and peak heart rate were significantly different from normal values showing an impaired functional capacity of these children. In conclusion we think that bidirectional cavopulmonary anastomosis can not be considered an adequate definitive palliation but it represents a very good stage to preserve the pulmonary arteries and to prepare the systemic ventricle towards the Fontan repair.

Adolescent

Bidirectional cavopulmonary anastomosis in patients under two years of age.

Between December 1986 and December 1990, a bidirectional cavopulmonary anastomosis was performed in 27 patients younger than 2 years of age, including 12 with heterotaxia syndrome. Age and weight of patients averaged 14.2 +/- 6.6 months and 8.1 +/- 2.2 kg, respectively. Eleven had pulmonary atresia and 16 had pulmonary stenosis. The main pulmonary artery was ligated in seven patients in the latter group (subsequently reopened in one) and left open in nine (subsequently ligated in two). There were four hospital deaths (15%). All patients were discharged with anticoagulant/antithrombotic therapy to be continued for 6 months. There were two late deaths before further operations (8.7%). Two patients, one with acquired massive pulmonary arteriovenous fistulas and one with progressive common atrioventricular valve regurgitation, subsequently underwent definitive repair (biventricular in one), and both died. Heterotaxia syndrome (p = 0.087) and preoperative mean pulmonary artery pressure higher than 15 mm Hg (p = 0.09) were the only risk factors for overall mortality.

Anastomosis, Surgical