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Biomedical subjects

C Marcelletti

Publications and source records attributed to C Marcelletti.

At least 55 records · Page 3Linked to original sources

Oral propafenone therapy for children with arrhythmias: efficacy and adverse effects in midterm follow-up.

The use of oral propafenone was assessed in 57 children. Mean age was 4.8 +/- 5.2 years (range 1 day to 17 years). Twenty-three patients were less than 1 year of age (40%) and 10 were less than 1 month of age (18%). Arrhythmias included supraventricular tachycardia in 32 patients (57%), atrial flutter in six (10%), and ventricular tachycardia in 19 (33%). Cardiac diagnoses included: an otherwise normal heart in 30 (53%); unoperated congenital heart defect in 11 (19%); cardiomyopathy in nine (16%); and an operated congenital heart defect in seven (12%). Mean dose of propafenone was 13.1 mg/kg/day (range 8 to 15). Success (assessed by relief of symptoms and serial 24-hour ECGs) was achieved in 16 of 32 patients with supraventricular tachycardia (50%), three of six with atrial flutter (50%), and 8 of 19 with ventricular tachycardia (42%). Drug efficacy in patients with and without organic heart disease was 30% (8 of 27) and 63% (19 of 30), respectively (p less than 0.05). Mean duration of therapy in patients in whom the drug was effective was 22.9 +/- 13.2 months (range 6 to 60). Growth was unimpaired in all patients. A neonate with nonsustained monomorphic ventricular tachycardia had sustained ventricular tachycardia during the second day of propafenone therapy at a dose of 13 mg/kg/day. Sustained ventricular tachycardia disappeared when the drug was discontinued. Another patient had an asymptomatic and transient increase in the glutamic pyruvic transaminase level. No adverse hemodynamic effects were observed. In conclusion, propafenone is an effective antiarrhythmic drug in children with normal hearts; it is less effective in patients with heart disease.(ABSTRACT TRUNCATED AT 250 WORDS)

Administration, Oral↗

Evaluation of the Damus-Kaye-Stansel operation in infancy.

Thirteen patients, 12 of whom younger than 2 years, underwent a Damus-Kaye-Stansel procedure for complete transposition of the great arteries, ventricular septal defect, or double-outlet right ventricle and subpulmonary ventricular septal defect. In 6 patients, associated cardiac anomalies caused systemic flow obstruction. There were six hospital deaths (mortality rate, 42%). In a mean follow-up period of 57 months, 5 of 7 survivors required relief of right ventricular hypertension through conduit replacement or enlargement (4 patients) or conduit valve balloon dilation (1 patient). The aortic valve became regurgitant in 2 patients in whom it had been left in potential connection with the right ventricle. One patient has moderate pulmonary valve regurgitation. The main advantage of the Damus-Kaye-Stansel procedure is that it avoids coronary relocation; also, the spatial relationship of the great arteries and the coronary anatomy do not affect its feasibility. One drawback is the need for a conduit in infancy. Our present indication for Damus-Kaye-Stansel procedure is confined to double-outlet right ventricle with subpulmonary ventricular septal defect; 5 of 6 patients survived repair in this series. Possible indications are for patients with associated subaortic obstruction or unusual coronary arrangements. Fresh or cryopreserved homografts as extracardiac conduits and primary closure of the subaortic area may reduce the need for reoperation after Damus-Kaye-Stansel procedure.

Blood Vessel Prosthesis↗

Pulmonary atresia with ventricular septal defect. Selection of patients for systemic-to-pulmonary artery shunt based on echocardiography.

From January 1987 to December 1988, in 22 infants with PAVSD, the diagnostic results obtained with echocardiography (two-dimensional, Doppler, and color) were prospectively compared to the angiocardiographic findings. We classified into group 1 patients with confluent and good-sized pulmonary (greater than or equal to 3 mm) arteries, single ductus arteriosus, and normal pulmonary venous connections ("favorable pattern"). The other patients with PAVSD were classified into group 2 ("unfavorable pattern"). The intracardiac anatomy, the morphology of the pulmonary arteries, and the pattern of pulmonary blood supply and pulmonary venous connection were correctly identified with echocardiography in all but one patient, who was erroneously considered to be in group 2. No false-positive of the "favorable pattern" (group 1) was detected. Echocardiography is an effective tool in infants with PAVSD, in order to discriminate cases with "favorable" and "unfavorable" patterns of pulmonary arteries, pulmonary blood supply, and pulmonary veins. The first group with the "favorable pattern" may be considered for systemic-to-pulmonary shunt surgery without angiocardiography. Based on this experience from January to December 1989, four patients with this "favorable pattern" underwent a successful systemic-to-pulmonary artery shunt with an echocardiographic diagnosis alone.

Angiocardiography↗

Di George anomaly with atrioventricular canal.

We report the first case of Di George anomaly associated with atrioventricular canal. This combination of anomalies may represent a chance occurrence of two situations happening in the same patient or, alternatively, the result of a single unknown embriogenetic mechanism.

DiGeorge Syndrome↗

Atrioventricular canal in Down syndrome. Prevalence of associated cardiac malformations compared with patients without Down syndrome.

The atrioventricular canal is the "classic" congenital heart anomaly in Down syndrome. We may learn more of the nature of this disorder by careful study of the anatomic characteristics of the cardiac lesions and by comparing these lesions in patients with and patients without Down syndrome. We reviewed the clinical characteristics (echocardiographic and angiocardiographic) of 220 patients with atrioventricular canal and compared the prevalence of anatomic types and associated cardiac malformations in children with (105) and without (115) Down syndrome. In patients with Down syndrome, the complete form of atrioventricular canal was prevalent, with a high frequency of associated Fallot's tetralogy. Partial atrioventricular canal and left-sided anomalies were more common in patients without Down syndrome. Down syndrome is associated with a simpler type of atrioventricular canal when compared with patients with a normal chromosome configuration.

Coronary Circulation↗

Total anomalous pulmonary venous connection: surgical repair with a double-patch technique.

A double-patch technique has been used to repair total anomalous pulmonary venous connection in 35 patients (mean age, 4.9 months; mean weight, 4.2 kg). In all patients with supracardiac or subdiaphragmatic type of total anomalous pulmonary venous connection, repair has been accomplished by enlarging the "new" left atrium. There were three early deaths (3 of 35 = 8.6%) and no late deaths in a mean follow-up of 30 months (3 to 71 months). We propose this double-patch technique as an alternative surgical option for total anomalous pulmonary venous connection.

Anastomosis, Surgical↗

Systemic atrioventricular conduit for extracardiac bypass of hypoplastic systemic atrioventricular valve.

The management of severe congenital mitral stenosis in infants and children is still controversial. We describe our experience with the use of a systemic atrioventricular (SAV) extracardiac conduit to bypass a hypoplastic systemic atrioventricular valve. An SAV extracardiac conduit has been used in six patients (left atrium--left ventricle in five, right atrium--right ventricle in one). One hospital death occurred due to mediastinitis and there were two late deaths, one due to progressive subaortic stenosis and one sudden, possibly due to arrhythmia. Postoperative cardiac catheterization performed in five patients showed reduction of the transmitral gradient from a mean of 16 mmHg to a mean of 5 mmHg. Calcification of the bioprosthetic valve occurred in two patients 3 1/2 years and 2 years respectively after the operation; one died from concomitant subaortic stenosis and one underwent conduit replacement. Although its long-term efficacy is limited, the SAV conduit seems the most reliable surgical option for infants and children with hypoplastic systemic atrioventricular valves unsuited to conventional surgery.

Aortic Coarctation↗

Pediatric cardiac surgery guided by echocardiography. Established indications and new trends.

Cardiac surgery in 602 children was not preceded by cardiac catheterization, the diagnosis being based on clinical findings and two-dimensional and Doppler echocardiography. In the 355 operations without cardiopulmonary bypass there were nine major and seven minor diagnostic errors (2.5% and 2%). Among the 247 cases with open-heart surgery there were no major and eight (3.2%) minor errors. The malformations most suitable for nonbypass surgery without catheterization seem to be those with reduced pulmonary blood flow requiring systemic-pulmonary artery shunt, aortic coarctation and patent ductus arteriosus. For open-heart surgery without invasive investigation, atrial septal defect, partial atrioventricular canal, aortic and pulmonary stenosis, cardiac tumor and isolated valve disorder are 'classic' candidates. Recent experience indicated that selected cases of complete atrioventricular canal, tetralogy of Fallot, truncus arteriosus, total anomalous pulmonary venous connection and transposition of the great arteries may safely undergo primary repair without cardiac catheterization. Because of its diagnostic potentialities, pediatric cardiac surgeons must become familiar with echocardiography.

Cardiac Catheterization↗

Inferior vena cava-pulmonary artery extracardiac conduit. A new form of right heart bypass.

From November 1988 to May 1989, four patients underwent total right heart bypass by means of bidirectional cavopulmonary anastomosis and interposition of an extracardiac conduit from the inferior vena cava to the pulmonary artery. All of them had an uneventful postoperative course, and there have been no early or late deaths. We propose this technique as an alternative surgical option in candidates for a Fontan procedure with (1) hypoplasia or atresia of the left atrioventricular valve, (2) common atrioventricular valve, (3) anomalies of systemic and pulmonary venous return, or (4) auricular juxtaposition.

Arteriovenous Shunt, Surgical↗

[Interventricular muscular defect in the atrioventricular canal].

The muscular ventricular septal defect associated with the atrioventricular canal is a malformation which has not yet been extensively studied. Between June 1982 and December 1989, 151 patients with atrioventricular canal underwent echocardiography and angiocardiography in our Department. Of these 95 (62.9%) had a complete form and 56 (37.1%) a partial. Among the 151 patients, 81 (53.6%) presented Down syndrome. We found 5 muscular ventricular septal defects in 4 patients; in 3 cases there was a single defect and in one case two defects. These defects were midmuscular in all patients and one patient also presented an apical defect. All 4 patients with muscular ventricular septal defect presented a complete atrioventricular canal and aortic coarctation; 3 out of 4 patients had a hypoplastic left ventricle with absence of Down syndrome. The muscular ventricular septal defect is a malformation which is rarely associated with atrioventricular canal (4/151 = 2.6%). In our experience, it was always associated with a complete form with aortic coarctation and was very rare in Down syndrome patients (1/81 = 1.2%). These findings may represent a peculiar association of anomalies which may be caused by fetal hemodynamic mechanisms.

Abnormalities, Multiple↗

[Hypothermia treatment of junctional ectopic tachycardia after surgical repair of congenital heart defects].

Junctional ectopic tachycardia after surgical repair of congenital heart defects is associated with high mortality. Usually, it is transitory and resolves spontaneously, but a long period with very fast heart rate and without atrio-ventricular synchrony contraction may cause low cardiac output. Treatment with common anti-arrhythmic drugs is often uneffective both in restoring sinus rhythm and in reducing heart rate. Since hypothermia is known to decrease cardiac automaticity, two infants, aged 4 and 10 months, with junctional ectopic tachycardia and low cardiac output after surgical repair of the atrio-ventricular septal defect were treated with hypothermia after unsuccessful pharmacological attempts to control the arrhythmia. Generalized hypothermia was induced with cooling-blankets and ice packs. Rectal temperature initially dropped to 30 degrees and was subsequently maintained at between 33.8 and 34.2 degrees with the heart rate between 130 and 140 m beats per minute. Trans-oesophageal atrial pacing at a higher rate allowed for sequential atrio-ventricular contraction. Signs of low cardiac output were quickly resolved. One patient was warmed-up after 12 hours of hypothermia and remained in sinus rhythm. In the other patient, the arrhythmia recurred after rewarming and a further 30-hour period of hypothermia was required. Sinus rhythm was maintained thereafter. Both patients are in stable sinus rhythm 20 and 22 months after surgery. Our experience supports the use of hypothermia as a means to control post-operative junctional ectopic tachycardia resistant conventional anti-arrhythmic drugs.

Electrocardiography↗

[Surgical correction of atrioventricular canal without heart catheterization].

In our department, 35 patients with atrioventricular canal underwent elective surgery between June 1983 and May 1989. The diagnosis was based on clinical and echocardiographic assessment without cardiac catheterization. Nineteen patients (age 2-12 years; mean 6.6 years) had a partial atrioventricular canal and 16 (age 5-8 months; mean 7 months) presented a complete atrioventricular canal. The echocardiographic diagnosis was confirmed at surgery in all but one patient (2.8%). The latter was diagnosed as having a complete canal but at surgery a transitional canal with a restrictive ventricular septal defect was found. We based our patient selection on the natural history which excluded cases with early heart failure and pulmonary hypertension. We also relied on an accurate morphological study using two-dimensional echocardiography which excluded cases with associated cardiac malformations. Our recent experience (1986-1989) showed that 50% of the patients with complete canal and 60% of patients with partial canal underwent surgery without cardiac catheterization.

Age Factors↗

Airway obstructions associated with congenital heart disease in infancy.

Significant airway obstruction may complicate the natural and surgical history of infants with congenital heart diseases. Airway obstruction occurred in 12 infants who had operations for congenital heart disease. In all cases tracheography demonstrated the cause of airway obstruction. Significant mortality (5/12, 41.7%) and morbidity in this group of infants were the result of airway obstruction. To reduce the complications caused by airway obstruction in infants with congenital heart disease, we recommend (1) preoperative identification of patients with potential airway obstruction, (2) preoperative tracheography in high-risk infants, (3) appropriate choice of the surgical procedure, especially when insertion of a prosthetic conduit is required, (4) early recognition of the problem during the postoperative period, (5) prompt diagnosis by postoperative tracheography, with or without angiography, and (6) therapy directed at the cause of airway obstruction.

Airway Obstruction↗