Surgery for congenital heart disease in the adult age

Despite congenital heart malformations are currently treated in infancy and childhood, a great number of patients still need surgical treatment in adult age. For this reason, we have embarked on a multicentric study involving 7 major italian centers (Padova, Milano S.Donato, Milano Niguarda, Bergamo, Bologna, Massa, Napoli), so as to evaluate the impact of cardiac surgery in adults with congenital heart disease in our country and survival determinants. In addition, clinical late morbidity was analyzed in order to to evaluate correlated pre-operative and operative risk factors. Methods We collected data of 856 patients who underwent 1179 procedures from January,1st 2000 to December 31st 2004. Patients were divided into three groups: Group I- Palliation (3.1%): any operation performed to improve patientâ's clinical status without restoring normal anatomy or physiology. Bidirectional cavopulmonary anastomosis and pulmonary artery banding were the most frequent procedures. Group II- Repair (69.7%): first operation performed in the patient, to achieve an anatomic or physiologic repair by separation of the pulmonary from systemic circulation (including also Fontan-types, and 1 and ’½ ventricle repairs). Most frequent procedures were: atrial septal defect closure (35.8%), partial anomalous pulmonary venous connection repair (7.2%), ventricular septal defect closure (5.3%). Group III- Reoperation (27.4%): all procedures performed after repair either anatomic or physiologic. The most frequent procedures were conduit replacement (9.8%), aortic (8.6%) or pulmonary valve replacement (7.7%) . Results Preoperatively 34.6% of patients were in NYHA class I, 48.4% in class II, 14.2% in class III and 2.8% in class IV. Sinus rhythm was present in 83%. There were 1179 procedure performed in 856 patients (1.37 procedure/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 pts (28.8%), with postoperative arrhythmias being the most frequent (26%). At mean follow-up of 22 months (range 1 month- 5.5 years), 86% of data were available. Late death occurred in 5 patients (0.5%). Patients were in NYHA class I in 79.3 % , II in 17.6%, III in 2.9%, and only one patient in class IV (0.11%). Ability index was class I in 82%, class II in 13.7% and class III in 2.3%. Overall survival estimates is 82.6% , 99% and 91.8% at 5 years for groups I, II, III respectively. Freedom from adverse events at 5 years is 91% for acyanotic vs 63.9 % for preoperative cyanotic patients (p < 0.0001). Multivariate Cox analysis identifies among the most powerful incremental risk factors for survival preoperative NYHA class IV in cyanotic patients (Hazard Ratio-HR- 8.6, p value 0.001), preoperative NYHA class III (HR 2.7, p value 0.023), and reoperation (HR 2.3, p value 0.029). In addition, multivariate Cox analysis for postoperative morbidity expressed as NYHA class greater than 1, identifies among the most powerful incremental risk factors the length of ICU stay (HR 1.037, CI=1.002-1.072, p=0.036), number of operations (HR 1,445 CI=1,1213-1,721, p<0.001), cyanosis (HR 1,555, CI1,035-2,335, p=0,034), alteration of cardiac rhythm before surgery (HR 1,124, CI=1,040-1,215, p=0,03), pre-operative NYHA class>1 (Hazard Ratio 1,573, CI=0,954-2,593, p=0.076), age > 40y (HR 1,466, CI1,014-2,119, p=0.042). Conclusions Surgery for congenital heart disease in adult age is a safe and a low risk treatment. However patients with preoperative cyanosis show a higher incidence of late non-fatal complications. In addition, better preoperative clinical conditions are correlated with better late clinical outcomes, thus early repair (before cardiac and non cardiac organ deterioration occurs) is advocated.