Minimally invasive or interventional repair of atrial septal defects in children: experience in 171 cases and comparison with conventional strategies.

OBJECTIVES: The goal of this study was to evaluate percutaneous interventional and minimally invasive surgical closure of secundum atrial septal defect (ASD) in children. BACKGROUND: Concern has surrounded abandoning conventional midline sternotomy in favor of the less invasive approaches pursuing a better cosmetic result and a more rational resource utilization. METHODS: A retrospective analysis was performed on the patients treated from June 1996 to December 1998. RESULTS: One hundred seventy-one children (median age 5.8 years, median weight 22.1 kg) underwent 52 device implants, 72 minimally invasive surgical operations and 50 conventional sternotomy operations. There were no deaths and no residual left to right shunt in any of the groups. The overall complication rate causing delayed discharge was 12.6% for minimally invasive surgery, 12.0% for midline sternotomy and 3.8% for transcatheter device closure (p < 0.01). The mean hospital stay was 2.8 +/- 1.0 days, 6.5 +/- 2.1 days and 2.1 +/- 0.5 days (p < 0.01); the skin-to-skin time was 196 +/- 43 min, 163 +/- 46 min and 118 +/- 58 min, respectively (p < 0.001). Extracorporeal circulation time was 49.9 +/- 10.1 min in the minithoracotomy group versus 37.2 +/- 13.8 min in the sternotomy group (p < 0.01) but without differences in aortic cross-clamping time. Sternotomy was the most expensive procedure (15,000 EUR +/- 1,050 EUR vs. 12,250 EUR +/- 472 EUR for minithoracotomy and 13,000 EUR +/- 300 EUR for percutaneous devices). CONCLUSIONS: While equally effective compared with sternotomy, the cosmetic and financial appeal of the percutaneous and minimally invasive approaches must be weighed against their greater exposure to technical pitfalls. Adequate training is needed if a strategy of surgical or percutaneous minimally invasive closure of ASD in children is planned in place of conventional surgery.

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.

The hypoplastic mitral valve. When should a left atrial-left ventricular extracardiac valved conduit be used?

Limited experimental and clinical experience with extracardiac bypass of the mitral valve has been reported. We describe the case history of a 3-year-old child in whom a left atrial-left ventricular valved conduit was successfully used to bypass a severely hypoplastic parachute mitral valve. The potential applications of this unconventional surgical option are reviewed.

Surgical bypass of the systemic atrioventricular valve in children by means of a valved conduit.

The surgical approach to relief of mitral stenosis in children is still a controversial problem. We describe our experience with four severely symptomatic children in whom a valved conduit from the left atrium to the left ventricle was successfully used to bypass a hypoplastic systemic atrioventricular valve. A left atrial-left ventricular extracardiac conduit was implanted in these patients with a hypoplastic mitral anulus and an adequate left ventricular chamber. There were no early or late deaths. Postoperative cardiac catheterization performed in all patients 1 month after the operation showed reduced size of the left atrium, a reduction of the left atrial-left ventricular gradient from a mean of 14 mm Hg to a mean of 5 mm Hg, and an increase of the left atrial outlet from a mean diameter of 10.7 mm to 28.7 mm (including the diameter of the native mitral valve plus the internal diameter of the valved conduit). The application of this unconventional operation in children with congenital or acquired stenosis of the systemic atrioventricular valve should be considered when the mitral valve obstruction cannot be relieved by conventional valve repair or replacement. Furthermore, the left atrial-left ventricular conduit does not preclude future alternative surgical options.

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.

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.

Bidirectional cavopulmonary shunts: clinical applications as staged or definitive palliation.

A standard Glenn anastomosis between the superior vena cava and the right pulmonary artery has been the accepted mode of treatment for patients with complex cyanotic congenital heart disease. We report our experience in 18 patients with such disease who underwent a bidirectional cavopulmonary shunt because of increasing cyanosis and growth cessation. All patients were considered less than "ideal" candidates for a Fontan procedure. We divided the patients into two groups: group 1 had azygos continuation and group 2 did not. Fourteen patients required hypothermic cardiopulmonary bypass. Bidirectional pulmonary blood flow was achieved in all patients. Only 1 death occurred (group 2). The improvement in oxygen saturation and overall clinical condition of these patients, together with the low mortality and morbidity, is encouraging. However, long-term follow-up is mandatory for a comprehensive evaluation of this surgical approach as definitive palliation or as a first stage for a Fontan operation.

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.

Right submammary minithoracotomy for repair of congenital heart defects.

OBJECTIVE: The initial experience with the right submammary minithoracotomy incision for correction of intracardiac congenital defects is reported. METHODS: Between March 1997 and March 1999, 100 children underwent repair of congenital heart disease through this approach. Their mean age and weight were 4.6 years and 20 kg, respectively. Diagnosis included: atrial septal defect (78), ventricular septal defect (7), tetralogy of Fallot (6), partial atrioventricular canal (5), double-chambered right ventricle (3) and single ventricle with dextrocardia (1). The standard technique entailed a 5 to 6 cm right submammary incision, entering the chest through the third or fourth intercostal space (depending on the body weight), direct aortic and bicaval cannulation and aortic cross-clamping with cardioplegic protection. RESULTS: There were no hospital deaths. Postoperative morbidity included bleeding in two cases, recurrent atrial septal defect in one, spleen injury in one. The average hospital stay was 3.5 days. All patient are currently free of symptoms and medications. CONCLUSIONS: (1) This approach for repair of selected congenital cardiac malformations is technically feasible, safe and effective; (2) younger age is a facilitating factor; (3) hospital stays are effectively reduced.

[Intraoperative stent implantation in congenital stenosis of pulmonary veins]. / Implianto intraoperatorio di "stent" nella stenosi congenita delle vene polmonari.

Congenital stenosis and/or hypoplasia of the pulmonary veins(s) is a rare cardiac malformation, whose treatment is difficult and not well defined, so conditioning an extremely poor prognosis. In fact, balloon angioplasty and surgery proved to be ineffective during a mid-term follow-up, thus not significantly modifying the natural history of this cardiovascular malformation. Although heart-lung transplantation is thought by someone to be the sole reliable treatment for congenital stenosis of the pulmonary vein(s), a recently available therapeutic option, the endovascular "stent" implantation during cardiac catheterization prompted to hope for a better outlook for these patients. However, this procedure is far from being an easy approach, due to technical problems that cause a high rate of failure and major complications. We report on two cases of pulmonary vein stenosis successfully treated by endoluminal "stent" implantation during open heart surgery, and suggest, and suggest that this safe and effective approach might be an alternative therapeutic option in the definitive or palliative treatment of this congenital cardiovascular malformation.

Simultaneous patency of ductus arteriosus and surgical shunt in pulmonary atresia with intact ventricular septum. A cause of acute myocardial failure?

Twenty-seven patients with pulmonary atresia and intact interventricular septum were treated with prostaglandin E1 infusion, followed by standard or modified Blalock-Taussig shunt. Three infants died. In all three there was anatomic and functional patency of the ductus arteriosus and the surgical systemic-pulmonary shunt. The unusual clinical course in these cases suggests that in the clinical management of patients with pulmonary atresia and intact interventricular septum after a shunt operation, particular attention must be paid to the problems associated with persistent ductal patency.

[Seroma: a complication of systemic-pulmonary shunt with a tubular prosthesis]. / Sieroma: una complicanza nello shunt sistemico-polmonare con protesi tubulari.

The accumulation of clear fluid leaking from a tubular prosthesis and confined within a fibrous pseudomembrane has been defined as a "seroma". Both the literature and our experience on this complication have been reviewed. Fifty-three cases of seroma have been reported after implantation of a tubular prosthesis. In our experience, from June 1982 to September 1988 we observed 11 cases (11/108 = 10.1%) of seroma complicating a modified Blalock-Taussig procedure. The mean age of these patients was 2 years 2 months. The diameter of the implanted tubular prostheses was always 5 mm. Among these cases, we report the details of a child, 1 year 10 months old, in whom the seroma significantly complicated the surgical history. According to the data derived from the literature and from our experience, we derived the following risk factors for the occurrence of seroma: age of the patient, size of the prosthesis, surgical manipulations, arterial-venous gradient, transprosthetic flow.

[What are the costs of pediatric cardiosurgery?]. / Quanto costa la cardiochirurgia pediatrica?

A cost analysis of patients discharged from the Department of Pediatric Cardiology and Heart Surgery of "Bambino Gesu" Children's Hospital was performed. Analysis focused on the costs and revenue from Diagnosis-Related Group 108 ("other cardiovascular procedures") during a six-month period in 1994 (n. = 76). The sample of 30 charts reviewed (MLOS 20.36 +/- 27.87 days) showed a mean cost of 30,381,000 italian lire (ITL) vs a revenue of ITL 23,545,800. The Pearson correlation coefficient between LOS and total cost was high (R = 0.85; p < 0.001). Other cases in DRG 108 (n. = 46) had an MLOS of 29.13 +/- 25.25 days and a higher cost. We emphasize the usefulness of identifying the costs for patients grouped in a specific DRG in order to establish an accurate departmental budget as well as to ensure the financial survival of referral hospitals.

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)

[Behavior of the blood calcium in operations during extracorporeal circulation]. / Comportamento del calcio ematico negli interventi in circolazione extracorporea.

The Authors analyse the variation of the calcium plasmatic concentration during extra corporeal circulation (E.C.C.). The Authors remind the importance of the calcium plasmatic concentration preserving a good cardiac output. They say that amount of calcium given assures a good plasmatic concentration and aid the cardiac output after E.C.C. The possible hypercalcemia after A.C.D. blood transfusion doesn't compromit the cardiovascular function.

[Extracorporeal circulation. Considerations on the methods adopted]. / La circolazione extracorporea. Considerazioni sulle metodiche adottate

This paper described the various types of procedures used for cardiopulmonary bypass related to the operative procedures. The Authors emphasize emodiluition and hypothermia as local as general recommend a limited use of coronary perfusion.

[Our experience with mitral valve surgery (author's transl)]. / La nostra esperienza nella chirurgia della valvola mitrale.

Our experience is based on 323 surgical operations on the mitral valve. Prosthetic valves were substituted in 308 patients and in 15 an anulo-valvulo-plastic operation. Mitral valve operation was associated with the correction of other heart lesions in 132 patients and in 197 patients, (objects of this study) surgery was carried out on the left atrio-ventricular valve. A detail report of the types of lesions found is made: 78 patients had massive calcification and 15 had a process of acute and subacute endocarditis. Mechanical prosthesis (Starr-Edwards 51; Björk-Shiley 77) were used 128 times and biologic prosthesis (Hancock 44; Carpentier-Edwards 7; Angell-Shiley) 54 times. Preference was given to the use of porcine prosthesis in patients presenting generic or specific controindications to anticoagulant therapy. Total mortality rate was 9,9% and in the last two years this fell to 5%. In our opinion, the sharp fall in mortality could be attributed to refined technic, better selection of cases, better myocardial protection consequent to the clinical use of cardioplegia and hypothermia.

[The subclavian-pulmonary anastomosis (author's transl)]. / L'anastomosi succlavia-polmonare oggi.

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.

[Interventricular muscular defect in the atrioventricular canal]. / Difetto interventricolare muscolare nel canale atrioventricolare.

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.

[Classification of congenital heart diseases and surgical interventions in pediatric cardiology for the standardization of hospitalization records]. / Classificazione delle cardiopatie congenite e degli interventi di cardiologia e cardiochirurgia pediatrica per una codifica standardizzata della documentazione di ricovero.

The utilization of DRGs for the evaluation of hospital activity requires the availability, on a current basis, of coded in-patient records (Hospital Discharge Form, SDO) with information concerning nosology of the patient and procedures performed. Consequently, the promotion of a standardized use of ICD.9 and ICD.9.CM code systems (International Classification of Diseases, 9th Revision, and International Classification of Diseases, 9th Revision, Clinical Modification) among clinicians is necessary. The National Research Council, Council Research Hospital for Cardiology and Cardiac Surgery (CREAS-IFC-CNR) has promoted the establishment of a permanent work group for the evaluation of the activity in the field of pediatric cardiology and cardiosurgery at a national level. In co-operation with two leading institution (AO "Bambino Gesù", Rome; AO "G. Gaslini", Genoa) an in-patient survey has been promoted including an overall revision of the ICD.9.CM code systems both for diagnosis and procedures in order to improve the standardization of data. Besides, the fitness of DRGs allocation by different codes has been judged. Some general and specific suggestions upon codes adequacy and DRGs identification have emerged from this study. The aim of this paper is to spread this preliminary standardization activity of the group as a contribution to the improvement of in-patient coded records quality.