The impact of native Fallot anatomy on future therapeutic requirements and outcomes at follow-up.

BACKGROUND: In patients with repaired Fallot, subsequent surgical or interventional procedures and adverse cardiac events are frequent. We aimed to evaluate the impact of a simple pre-operative anatomic classification based on the size of the pulmonary valve (PV) annulus and branches on future therapeutic requirements and outcomes. METHOD: This is a single-center retrospective analysis of patients operated for Fallot before the age of 2 years, from January 1990. Pre-operative anatomy, surgical and interventional procedures and adverse events were extrapolated from clinical records. RESULTS: Among the 312 patients, a description of the PV and pulmonary arteries (PAs) native anatomy was known in 239 patients (male:147, 61.5%), which were divided in the following 3 groups: group 1 (65 patients) with normal size of both PV and PAs; group 2 (108 patients) with PV hypoplasia but normal size PAs; group 3 (66 patients) with concomitant hypoplasia of the PV and PAs. During the 12.7 years (IQR 6.7-17) follow-up time, 23% of patients required at least one surgical or interventional procedure. At Kaplan-Meier analysis, there was a significant difference in requirement of future surgical or interventional procedures among the 3 groups (p < 0,001). At multivariate Cox regression analysis, hypoplasia of PV and PAs was an independent predictor of subsequent procedures (HR:3.1,CI:1.06-9.1, p = 0.03). CONCLUSION: Native anatomy in Tetralogy of Fallot patients affects surgical strategy and follow-up. It would be therefore advisable to tailor patient's counseling and follow-up according to native anatomy, rather than following a standardized protocol.

[The project "Don Carlo Gnocchi Foundation--Cardio-Thoracic Department De Gasperis--Lombardy Region" for the care of children from socio-economically deprived areas affected by cardiac disease]. / II progetto "Fondazione Don Carlo Gnocchi--Dipartimento Cardiochirurgico De Gasperis--Regione Lombardia" per la cura di bambini cardiopatici provenienti da paesi esteri a basso livello assistenziale.

BACKGROUND: In 2001 the Cardiac Rehabilitation Unit IRCCS S. Maria Nascente Center and the International Area of Don Carlo Gnocchi Foundation, in collaboration with the Cardiac Surgery Department "De Gasperis" of Niguarda Ca' Granda Hospital in Milan, planned a project to treat children from impoverished countries. The "Fondo Sanitario Regionale" of the Lombard Region cosponsored the program. METHODS: From October 2001 to November 2006, 32 patients (25 from Zimbabwe and 7 from Albania) were selected and submitted to cardiac surgery: 22 patients were affected by acquired valvular heart disease in NYHA class III-IV, 10 by congenital heart disease. After surgery the patients admitted to our rehabilitation unit underwent a period of comprehensive cardiac rehabilitation. Afterwards, the patients were in the care of selected Italian families for about 3 months. In both populations the problems faced in the selection, management and surgical approach are discussed. RESULTS: At 21 months the survival of the whole study population was 93 % (2 valvular patients died during the follow-up); 2 patients who initially underwent mitral valve repair were submitted to valve replacement for late appearance of severe regurgitation. In 3 patients with mitral valve bioprosthesis a significant structural valve deterioration occurred in the follow-up and 2 of them underwent valve replacement. CONCLUSIONS: The advantage of the excellent performance in durability of mechanical prosthetic valves (with respect to the limited durability of porcine bioprostheses), the problems with long-term anticoagulation have to be taken into consideration in the management of patients coming from socio-economically deprived areas.