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Biomedical subjects

C Marcelletti

Publications and source records attributed to C Marcelletti.

At least 109 records · Page 6Linked to original sources

Cardiac rhabdomyoma: surgical treatment in the neonate.

Primary cardiac tumors are rare. Of the benign lesions, rhabdomyoma predominates in infants and children. The natural history of patients with cardiac rhabdomyoma is poor. About 50% die in the first month of life and 80% by 1 year of age. We report on the two youngest infants ever operated upon successfully for removal of symptomatic ventricular cardiac rhabdomyoma. The operations were performed through a left ventriculotomy in one and through a right ventriculotomy in the other during the first day of life.

Cardiac Output, Low↗

Right and left isomerism: the cardiac surgeon's view.

We have treated by palliative or corrective surgery 12 patients with the complex cardiovascular anomalies associated with right isomerism (asplenia syndrome) or left isomerism (polysplenia syndrome). A systemic-pulmonary artery shunt was performed in 4 patients, with 2 early deaths. Anatomical intracardiac repair was successfully done in 2 patients, while an orthoterminal repair using a modified Fontan procedure was attempted in 5 patients, only 1 of whom survived. A 1-year-old girl with diffuse pulmonary arteriovenous fistulas associated with left isomerism was not considered a good candidate for operation after an exploratory sternotomy. The surgical options and technical problems related to the complex intracardiac morphology of these developmental syndromes are presented.

Adolescent↗

The Fontan procedure in the absence of the interatrial septum. Failure of its principle?

The Fontan principle, defined as a procedure in which the right ventricle is bypassed in order to convey desaturated venous blood from the right atrium to the lungs, is presently applied for a wide variety of congenital heart malformations including those in which there is no suitable ventricular pumping chamber. Recently, the procedure has also been advocated for complex malformations that require atrial septation or intra-atrial rerouting. The present report evaluates our experience in four patients with such complex malformations. Three had a complete form of atrioventricular septal defect with double-outlet right ventricle and one patient had left atrioventricular valve atresia. The common atrium was morphologically right in two patients and morphologically left in one. In each of these instances anomalous pulmonary venous connections were present, together with abnormal systemic venous connections. The results were unsatisfactory. Three of the four patients died. The only survivor had no pulmonary or systemic venous abnormalities. Severe impairment of pulmonary blood flow was one of the most important postoperative complications. The findings suggest that the complexities of rerouting the blood within the atria play an important role. The excess proportion of prosthetic material to atrial myocardium may result in excessive loss of contractile atrial myocardium and, probably equally significant, in a complex intra-atrial geometry of pathways that may cause a critical pressure gradient. The results clearly show that with an expanding horizon of the application of the Fontan principle, new pitfalls may arise which presently appear to be the prevailing factors limiting its success.

Adolescent↗

Univentricular heart: can we alter the natural history?

Surgical treatment must be considered for patients with univentricular heart in view of their poor natural history. Since one of the major factors influencing the natural history of this malformation is the amount of pulmonary blood flow, we discuss the potential surgical options by separately considering the two main pathophysiological situations: univentricular heart with restricted pulmonary blood flow, and univentricular heart with unrestricted pulmonary blood flow. We have reviewed the early and late results of surgical treatment based on our experience with 19 patients and the data from the literature. Temporary relief of symptoms can be provided by palliative operations (systemic-pulmonary shunt, atrioseptectomy, enlargement of the outlet foramen, pulmonary artery banding, or palliative Mustard or Senning procedure). "Corrective" surgery, by means of either a modified Fontan operation or ventricular septation, carries a high early mortality (about 30%) and a high early and late morbidity, with only 50% of survivors enjoying an asymptomatic life. Studies of the long-term efficacy of palliative operations as well as our experience and that of others with "corrective" operations, which have a relatively short follow-up, do not yet indicate whether presently available surgical procedures can alter the natural history of patients with univentricular heart.

Adolescent↗

Simplified thoracic approach to the ascending and descending aorta in complex coarctation.

Two patients with left ventricular and proximal aortic hypertension secondary to diffuse hypoplasia of the aortic arch have recently been treated by a surgical technique based on: I simultaneous exposure of the ascending and descending thoracic aorta through a left antero-latero-posterior thoracotomy; 2 insertion of a Dacron graft between the aortic root and the descending thoracic aorta. In both cases equal systemic pressures in the ascending and descending thoracic aorta and adequate distal perfusion were obtained.

Aortic Coarctation↗

Corrected transposition and ventricular septal defect. Surgical experience.

The entire 21-year experience at our institution with intracardiac repair of corrected transposition of the great arteries (CTGA) associated with ventricular septal defect (VSD) is reviewed. There were 53 such operations. The hospital mortality rate fell from 50% before 1972 to 18% since then (p < 0.02), and was 19% when a systemic ventriculotomy was not used compared to 60% when it was used (p < 0.01). A technique is described for correction of CTGA with VSD and pulmonary outflow tract obstruction (POTO) designed to minimize risk of heart block and to increase the degree of relief of POTO: the VSD patch is placed to the right side of the conduction tissue and the pulmonary outflow tract, and POTO is bypassed with an extracardiac conduit. This technique reduced the incidence of complete heart block from 67% by direct relief of POTO to 28% (p < 0.05) and decreased the frequency of inadequate relief of POTO from 50% to 8% (p < 0.01). However, experience with these two approaches was not concurrent. Successful intraoperative mapping of the conduction bundle was associated with operatively induced heart block in 37%, whereas prior to the availability of mapping the incidence of heart block was 57% (difference not significant). Twelve patients required atrioventricular (AV) valve repair or replacement, and in them the operative mortality was 50%, compared to 20% in the remainder (p < 0.05). For the 21 patients with VSD and POTO who achieved good relief of POTO, the operative mortality was only 5%. These findings suggest that good progress has been made in the surgical repair of CTGA with VSD, though further improvement in results is required.

Adolescent↗

Fontan's operation for Ebstein's anomaly.

Reported herein is the case of a patient with an almost imperforate form of Ebstein's anomaly of the tricuspid valve and a small muscular ventricular septal defect through which the left ventricle communicated with the right ventricular outflow tract. This particular anatomy facilitated the successful execution of a Fontan operation. We are of the opinion that this operation could find its place among the surgical options for Ebstein's anomaly.

Adult↗

Fontan's operation: an expanded horizon.

The Fontan principle of redirecting systemic venous blood into the pulmonary arteries via a conduit has now gained a much wider application than initially intended. We have applied this type of operation in a series of 15 patients, five of whom had "classical" tricuspid atresia with concordant arterial connections. The other 10 patients showed a variety of congenital malformations, such as double-inlet univentricular heart with discordant arterial connections, double-outlet right ventricle with common atrioventricular canal. Ebstein's anomaly with almost imperforate tricuspid valve, hypoplasia of right ventricular inflow, and a crisscross heart with straddling right atrioventricular valve. Important associated anomalies, such as common atrium and anomalous pulmonary and/or systemic drainage, required particular adaptation of the technique. Thirteen of the 15 patients survived operation and are presently alive. One death occurred in a patient in whom the preoperative diagnosis had to be adjusted during operation because of unexpected total anomalous pulmonary venous connections. The other death occurred in a patient suffering from the "asplenic syndrome." The autopsy in this patient suggested that the large baffle used for atrial septation and ventricular exclusion had obstructed pulmonary venous return. Our experience thus supports the opinion that Fontan's operation has a place in the treatment of otherwise anatomically uncorrectable lesions.

Adolescent↗

[The subclavian-pulmonary anastomosis (author's transl)].

A series of 16 patients who underwent Blalock-Taussig anastomosis for severe cyanosis, without hospital deaths, is reported. Based on this recent, limites experience, and on/an extensive review of the literature the Authors analyze objective criteria to identify the ideal palliative systemic-pulmonary shunt. Early results and late sequelae of Blalock-Taussig, Waterston-Cooley and Potts-Smith anastomosis are compared. It is then proposed that the subclavain-pulmonary anastomosis should be considered the palliation of choice of elective and emergency indication to treat severe cyanosis in those patients for whom corrective surgery can not be contemplated.

Adult↗

Palliative Mustard or Rastelli operation in complete transposition of the great arteries. Option decided by lung biopsy.

A case history is reported of a patient with complete transposition of the great arteries and a ventricular septal defect in whom pulmonary hypertension developed, with a pulmonary resistence of 10 units M.2. Because of the possibility that the pulmonary vascular obstructive disease might have been of recent onset and that a high hematocrit value of 82 percent may have interfered with the precise determination of pulmonary resistance, an open lung biopsy was performed. The histologic findings suggested that the vascular disease was as yet reversible. Because of these findings and the aforementioned considerations, a Rastelli operation was performed instead of a palliative Mustard procedure. At present, 6 months postoperatively, the patient is asymptomatic.

Adolescent↗

[Hypoplasia of left pulmonary artery secondary to aorta-right pulmonary artery anastomosis. Diagnostic and therapeutical considerations (author's transl)].

Three cases of tetralogy of Fallot treated under twelve months of life with aorta-right pulmonary artery anastomosis are reported, in which severe hypoplasia of left pulmonary artery secondary to palliation was present. In the first two cases this complication was not recognized during investigation prior to repair and both died of low cardiac output due to severe right ventricular hypertension. In the third case the complication was evidenced and treated with interposition of a prosthetic valved conduit between right ventricle and confluence of the pulmonary arteries. Hypoplasia or atresia of left pulmonary artery and right pulmonary vascular obstructive disease, secondary to a long-functioning aortopulmonary anastomosis, require surgical consideration identical to tetralogy of Fallot with single pulmonary artery, which is corrected with the use of a valved conduit. The Authors suggest that aortopulmonary shunts should be dismantled within two years and investigation prior to repair should include: 1) exploring of all cardiac chambers and right pulmonary artery through the fistula; 2) injection of contrast medium in right pulmonary artery, aortic root, right ventricle or pulmonary trunk. These diagnostic methods should demonstrate continuity between right ventricle and pulmonary arteries, confluence of pulmonary arteries, and rule out acquired left pulmonary artery atresia, right pulmonary vascular obstructive disease, right ventricular outflow tract atresia.

Aorta↗