"Baby Heart Project": the Italian project for accreditation and quality management in pediatric cardiology and cardiac surgery.

Optimization of the relationship between the supply and the demand for medical services should ideally be taken into consideration for the planning within each national Health System. Although government national health organizations embrace this policy specifically, the contribution of expert committees (under the scientific societies' guarantee in any specific medical field) should be advocated for their capability to collect and analyze the data reported by the various national institutions. In addition, these committees have the competence to analyze the need for the resources necessary to the operation of these centers. The field of pediatric cardiology and cardiac surgery may represent a model of clinical governance of particular interest with regard to programming and to a definition of the quality standards that may be extended to highly specialized institutions and ideally to the entire Health System. The "Baby Heart Project," which represents a model of governance and clinical quality in the field of pediatric cardiology and cardiac surgery, was born from the spontaneous aggregation of a committee of experts, supported by duly appointed Italian Scientific Societies and guided by a national agency for accreditation. The ultimate aim is to standardize both procedures and results for future planning within the national Health System.

Scimitar syndrome: a European Congenital Heart Surgeons Association (ECHSA) multicentric study.

BACKGROUND: Scimitar syndrome is a rare congenital heart disease. To evaluate the surgical results, we embarked on the European Congenital Heart Surgeons Association (ECHSA) multicentric study. METHODS AND RESULTS: From January 1997 to December 2007, we collected data on 68 patients who underwent surgery for scimitar syndrome. Primary outcomes included hospital mortality and the efficacy of repair at follow-up. Median age at surgery was 1.4 years (interquartile range, 0.46 to 7.92 years). Forty-four patients (64%) presented with symptoms. Surgical repair included intraatrial baffle in 38 patients (56%; group 1) and reimplantation of the scimitar vein onto the left atrium in 21 patients (31%; group 2). Eight patients underwent right pneumectomy, and 1 had a right lower lobe lobectomy (group 3). Four patients died in hospital (5.9%; 1 patient in group 1, 2.6%; 3 patients in group 3, 33%). Median follow-up time was 4.5 years. There were 2 late deaths (3.1%) resulting from severe pulmonary arterial hypertension. Freedom from scimitar drainage stenosis at 13 years was 83.8% in group 1 and 85.8% in group 2. Four patients in group 1 were reoperated, and 3 patients (2 in group 1 [6%] and 1 in group 2 [4.8%]) required balloon dilation/stenting for scimitar drainage stenosis. CONCLUSIONS: The surgical treatment of this rare syndrome is safe and effective. The majority of patients were asymptomatic at the follow-up control. There were a relatively high incidence of residual scimitar drainage stenosis that is similar between the 2 reported corrective surgical techniques used.

Surgical treatment of arrhythmias related to congenital heart diseases.

BACKGROUND: Supraventricular arrhythmias complicate operated and unoperated congenital heart disease, especially when atrial dilatation coexists. METHODS: We evaluated the results of intraoperative ablation in a group of 23 patients with chronic supraventricular tachyarrhythmias (mostly intraatrial reentry) that were unresponsive to conventional medical therapy. All procedures were performed consecutively between September 1999 and November 2001. Ablation was done during redo operations (Fontan conversion to total cavopulmonary connection in 16 patients) in 18 patients and during primary surgical correction in 5 patients. The mean age at operation was 25 +/- 12 years (2 to 50 years). Cryoablation was done in 10 patients and radiofrequency ablation in 13 patients. Nineteen patients had ablation in one atrium and 4 had ablation in both atria. A generous atrial reduction was always performed at the end of the operation. RESULTS: The operative mortality rate was 13% (3 patients) from causes unrelated to ablation. In 20 survivors, the ablation was effective immediately. Eight patients required a permanent pacemaker. During a mean follow-up of 22 +/- 4 months, atrial arrhythmias recurred in 25% (5 patients) and were controlled with medical therapy, whereas 1 patient required pacemaker implantation. CONCLUSIONS: Intraoperative treatment of unresponsive atrial tachyarrhythmias associated with operated or unoperated congenital heart disease is feasible and the midterm results are encouraging.

Unusual complication of arterial switch operation: ventricular septal aneurysm treated with surgical ventricular restoration.

Despite current low overall perioperative mortality, ischemic complications due to coronary artery translocation remain the most unwanted early complication of the arterial switch operation. We describe the case of a boy who underwent, at five days of age, one-stage arterial switch operation for transposition of the great arteries with repair of ventricular septal defect and coarctation of the aorta. Early postoperative course was complicated by severe ischemic left ventricle (LV) dysfunction and development of a ventricular septal aneurysm. At four years of age he underwent successful surgical ventricular restoration associated with implantation of a biventricular pacing, with complete resolution of heart failure symptoms.

An observational study of CoSeal for the prevention of adhesions in pediatric cardiac surgery.

We sought to evaluate the utility and safety of CoSeal Surgical Sealant (Baxter) for the prevention of cardiac adhesions in children. Seven cardiac surgery centers in Europe recruited consecutive pediatric patients requiring primary sternotomy for staged repair of congenital heart defects. Exclusion criteria included immune system disorder, unplanned reoperation, or reoperation within three months of primary repair. CoSeal was sprayed onto the surface of the heart at the end of surgery. Evaluation of adhesions took place at first reoperation. Data on safety, duration of surgery, and ease of CoSeal use were also collected. Seventy-nine pediatric patients were recruited between February 2005 and September 2007. Of these, 76 underwent major surgery to repair a wide range of congenital heart defects. Thirty-six patients underwent reoperation >3 months after primary repair, and were included in the efficacy analysis. Mean adhesions score was 8.3 (standard deviation [S.D.] 2.4; range 7-16). Six adverse events (5 serious) were possibly/definitely attributed to CoSeal. CoSeal's ease of use at primary operation was graded by surgeons as 12.1 mm (S.D. 9.8) on a visual analog scale of 0 ('very easy') to 100 mm ('very difficult'). Results of this prospective uncontrolled trial justify further investigation in a randomized, controlled trial.

The scimitar syndrome: an Italian multicenter study.

BACKGROUND: Scimitar syndrome is a rare congenital heart disease. To evaluate the results of managing this malformation surgically, we have embarked on a multicenter Italian study involving seven different centers and reporting the largest published series in the medical literature. METHODS: From January 1997 to December 2007, 26 patients with scimitar syndrome who underwent surgical correction were included. Primary outcomes include hospital mortality and the efficacy of repair at the follow-up. RESULTS: Median age was 11 years (interquartile range, 1.8 to 19.9 years). Nineteen patients (73%) presented with symptoms including upper respiratory tract infections (n = 13), recurrent pneumonia (n = 10), cardiac failure (n = 4), and cyanosis (n = 2). Associated cardiac anomalies were present in 16 patients (63%). Surgical repair included intraatrial baffle repair in 18 patients (69%; group 1), and reimplantation of the "scimitar vein" onto the left atrium in 8 patients (31%; group 2). One patient died in hospital (3.8%; group 1). Postoperative complications were less frequently reported in group 1 (4 of 18 patients, 22%) compared with group 2 (5 of 8 patients, 62%). Median follow-up time was 4 years (interquartile range, 1.8 to 9.7 years). There was 1 late death (1/25 patients, 4%; group 2). Four patients (16%) showed a complete occlusion of the scimitar drainage (2 in group 1, 12%; 2 in group 2, 25%) and 3 patients (12%) required balloon dilation or stenting for scimitar vein stenosis (1 in group 1, 6%; 2 in group 2, 25%). CONCLUSIONS: The intraatrial baffle repair seems to have a lower incidence of postoperative complications and a better patency rate, at last follow-up, than the reimplantation of the scimitar vein onto the left atrium.

Previous palliation in patients with tetralogy of Fallot does not influence the outcome of later repair.

OBJECTIVE: Primary repair is the treatment of choice in patients with tetralogy of Fallot. The timing of repair, however, remains controversial, and an initial palliative procedure might be considered a valuable option in the early management of symptomatic young infants and in those with either unfavourable anatomy, major associated lesions or chromosomal abnormalities with a poor life expectancy. METHODS: We reviewed the management of 100 consecutive patients with tetralogy of Fallot who were operated upon at our department during an 8-year period from June 1995 to March 2003. The rationale for the choice of the initial management and the outcome in terms of morbidity and mortality in patients who underwent primary repair was compared to that observed in patients who had had a two-stage repair. RESULTS: Age less than 3 months, the presence of either an unfavourable anatomy or major associated defects and genetic disorders with poor life expectancy were the indications for an initial palliation, which was carried out in 31 patients. There were no hospital deaths, and 28 of these patients underwent later repair with one hospital death (3.5%). Two patients with severe chromosomal abnormalities died at home and the remainder required a further palliation because of severely hypoplastic pulmonary arteries. Primary repair was carried out in 69 patients with one hospital death (1.4%). A transannular patch, which was used in 80% of our patients, was not an incremental risk factor for death regardless of the type of repair. Eight patients were reoperated on because of either residual right ventricular outflow tract obstruction with (four patients) or without (one patient) residual ventricular septal defect or isolated residual ventricular septal defect (three patients). All reoperations occurred in patients undergoing primary repair. CONCLUSIONS: The outcome of patients undergoing repair of tetralogy of Fallot is not influenced in terms of either mortality or morbidity by an initial palliative procedure.

Clinical, anatomopathological and genetic pattern of 10 patients with cervical aortic arch.

Cervical aortic arch (CAA) is a rare congenital abnormality in which the aortic arch is situated cranially to its usual position, often associated with complex developmental alteration of aortic laterality and branching. More recently, some authors reported deletion of chromosome 22q11 (del22q11) in few patients with CAA. The aim of this study was to describe the clinical, anatomopathological and genetic pattern of 10 new cases of CAA. From 1975 to 2003, 10 patients with CAA (4 female, mean age 12.4+11.1 years) underwent complete cardiovascular evaluation and screening for del22q11 using fluorescence in situ hybridization. Six patients underwent CAA surgical repair with histological analysis of aorta samples. Six patients were asymptomatic; one patient had syncope on effort, 2 patients had dyspnea on effort and 1 had cyanosis and dyspnea (tricuspid atresia). A murmur and/or a pulsatile mass of the neck was present in all patients. Femoral pulses were weak or absent in 6 patients. Four patients had right and 6 left CAA; kinking or true obstruction of the aortic arch was present in 9/10. Abnormal aortic arch branching pattern was found in 8/10 (mainly aberrant subclavian artery). Two patients had del22q11 with dysmorphic features, multiorgan anomalies and degenerative mediocystic necrosis of aortic wall. Our data confirm clinical polymorphism and anatomic complexity of CAA as well as the association between del22q11 and CCA in 2 syndromic patients.

Midterm results of surgical intervention for congenital heart disease in adults: an Italian multicenter study.

OBJECTIVE: We have analyzed, in a clinical multicenter study, the effect of cardiac surgery in adults with congenital heart disease in Italy. METHODS: We collected clinical data from 856 patients aged 19 years or older who underwent surgical intervention from January 1, 2000, to December 31, 2004. Patients were divided into 3 surgical groups: group 1, palliation (3.1%); group 2, repair (69.7%); and group 3, reoperation (27.4%). RESULTS: Preoperatively, 34.6% of patients were in New York Heart Association class I, 48.4% were in class II, 14.2% were in class III, and 2.8% were in class IV. Sinus rhythm was present in 83%. There were 1179 procedures performed in 856 patients (1.37 procedures per patient), with a hospital mortality of 3.1%. Overall mean intensive care unit stay was 2.3 days (range, 1-102 days). Major complications were reported in 247 (28.8%) patients, and postoperative arrhythmias were the most frequent. At a mean follow-up of 22 months (range, 1 month-5.5 years; completeness, 87%), late death occurred in 5 (0.5%) patients. New York Heart Association class was I in 79.3%, II in 17.6%, and III in 2.9%, and only 1 (0.11%) patient was in class IV. Overall survival estimates are 82.6%, 98.9%, and 91.8% at 5 years for groups 1, 2, and 3, respectively. Freedom from adverse events at 5 years is 91% for acyanotic patients versus 63.9% for preoperative cyanotic patients (P < .0001). CONCLUSIONS: Surgical intervention for congenital heart disease in adults is a safe and low-risk treatment. However, patients presenting with preoperative cyanosis show a higher incidence of late adverse events and complications.

Atresia of the common pulmonary vein.

A newborn girl with atresia of the common pulmonary vein, presented immediately after birth with severe cyanosis and acidosis. The diagnosis of totally obstructed total pulmonary venous return was made by cross-sectional echocardiography. Subsequent cardiac catheterization failed to demonstrate the site of pulmonary venous return. Necropsy showed the pulmonary veins to be connected bilaterally to an atretic common pulmonary vein. There was no obvious alternative pathway for pulmonary venous return.