Long-Term Survival Is Superior in Patients With Pulsatile Pulmonary Flow After the Björk Procedure.

Background: This study aims to evaluate clinical outcomes and hemodynamic variables late after the Björk procedure, regarding the pulmonary flow pattern. Methods: Patients who survived more than 15 years after the Björk procedure were included and then divided into two groups according to their pulmonary flow pattern by pulsed-wave Doppler assessment of echocardiography: patients with pulsatile systolic pulmonary flow (Group P) and those without (Group N). Results: A total of 43 patients were identified, of whom 13 patients were divided into Group P and 30 in Group N. Median age at the Björk procedure was 5.7 (2.1-7.3) years, and median follow-up was 32 (28-36) years. Survival after 15 years was higher in Group P, compared with Group N (100% vs 76% at 30 years, P = .045). Cardiac catheterization data demonstrated higher cardiac index in Group P patients compared with Group N patients (3.5 vs 2.8 L/m2, P = .014). Cardiac magnetic resonance imaging study revealed that Group P patients had higher right ventricular end-diastolic volume index (96 vs 57 mL/m2, P = .005), higher end-systolic volume index (49 vs 30 mL/m2, P = .013) and higher right ventricular stroke volume index (48 vs 25 mL/m2, P < .001), compared with Group N patients. Exercise capacity tests demonstrated that Group P patients showed a higher percent predicted peak oxygen consumption, compared with Group N patients (73 vs 58%, P < .001). Conclusions: Late after the Björk procedure, patients with a pulsatile systolic pulmonary flow had a larger right ventricle and better exercise capacity compared with those without pulsatile systolic pulmonary flow.

Impact of pulsatile pulmonary blood flow on cardiopulmonary exercise performance after the Fontan procedure.

Objective: To evaluate the exercise capacity in patients following Fontan-Kreutzer, Fontan-Björk, and total cavopulmonary connection (TCPC). Methods: Patients who performed exercise capacity tests at least once after the Fontan procedure between 1979 and 2007 were included. Patients after Fontan-Björk procedure were divided into 2 groups according to the pulmonary blood flow (PBF) pattern: patients with pulsatile PBF and those without. Peak oxygen uptake (VO2) was measured and percent-predicted VO2 was calculated. Results: A total of 227 patients were nominated. The types of Fontan procedure included Fontan-Kreutzer in 48 (21.1%) patients, Fontan-Björk in 38 (16.7%); 11 (4.8%) with pulsatile PBF and 27 (11.9%) without pulsatile PBF; and TCPC in 141 (62.1%). Median age at the Fontan procedure was 4.5 years (interquartile range, 2.1-8.2 years). A total of 978 cardiopulmonary exercise tests were performed at median follow-up of 17.7 years (interquartile range, 11.3-23.4 years) postoperatively. Analysis using linear mixed-effects models demonstrated that percent-predicted VO2 was greater in patients with pulsatile PBF after Fontan-Björk compared with patients after other types of Fontan procedure (P < .001). The same results were obtained when the longitudinal percent predicted VO2 was performed using only patients with tricuspid atresia and double inlet left ventricle (P < .001). Conclusions: Among long-term survivors after various types of Fontan procedures, patients with pulsatile PBF after the Fontan-Björk procedure demonstrated better exercise performance compared to those after TCPC, those after the Fontan-Kreutzer procedure, and those after the Fontan-Björk procedure with non-pulsatile PBF. The results implicate the importance of pulsatile PBF to maintain the Fontan circulation.