Twenty-year experience with heart transplantation for infants and children with restrictive cardiomyopathy: 1986-2006.

Idiopathic restrictive cardiomyopathy (RCM) is a rare cardiomyopathy in children notable for severe diastolic dysfunction and progressive elevation of pulmonary vascular resistance (PVR). Traditionally, those with pulmonary vascular resistance indices (PVRI) >6 W.U. x m(2) have been precluded from heart transplantation (HTX). The clinical course of all patients transplanted for RCM between 1986 and 2006 were reviewed. Preoperative, intraoperative and postoperative variables were evaluated. A total of 23 patients underwent HTX for RCM, with a mean age of 8.8 +/- 5.6 years and a mean time from listing to HTX of 43 +/- 60 days. Preoperative and postoperative (114 +/- 40 days) PVRI were 5.9 +/- 4.4 and 2.9 +/- 1.5 W.U. x m(2), respectively. At time of most recent follow-up (mean = 5.7 +/- 4.6 years), the mean PVRI was 2.0 +/- 1.0 W.U. x m(2). Increasing preoperative mean pulmonary artery pressure (PA) pressure (p = 0.04) and PVRI > 6 W.U. x m(2) (chi(2)= 7.4, p < 0.01) were associated with the requirement of ECMO postoperatively. Neither PVRI nor mean PA pressure was associated with posttransplant mortality; 30-day and 1-year actuarial survivals were 96% and 86%, respectively. Five of the seven patients with preoperative PVRI > 6 W.U. x m(2) survived the first postoperative year. We report excellent survival for patients undergoing HTX for RCM despite the high proportion of high-risk patients.

Sex-based differences in serum cardiac troponin I, a specific marker for myocardial injury, after cardiac surgery.

BACKGROUND: Prevalence and causes of sex-based differences in morbidity and mortality secondary to cardiovascular disease remain controversial. Cardiac troponin I (cTnI) is a sensitive and specific marker for myocardial injury. Serial cTnI measurements have been used to identify perioperative myocardial cell injury. OBJECTIVE: To determine whether sex influences the extent of myocardial injury during cardiac surgery, we measured perioperative cTnI in male and female patients. DESIGN: A total of 17 male and 17 female patients were prospectively studied in an age- and case-matched manner. Arterial cTnI were obtained preinduction, 30 mins after the application of the aortic cross-clamp, at arrival to the intensive care unit, and on postoperative day 1. SETTING: Tertiary cardiac surgery center at a major teaching hospital. RESULTS: There was no difference between men and women in body mass index (kg/m2), duration of cardiopulmonary bypass, and aortic cross-clamp times. Preoperative cTnI measurements were similar in men (0.24 +/- 0.15 ng/mL) and women (0.25 +/- 0.13 ng/mL, mean +/- sem). The maximum serum cTnI occurred on postoperative day 1 in all patients, and it was 3-fold higher in men (18.5 +/- 5.7 ng/mL) compared with women (6.4 +/- 1.0 ng/mL). CONCLUSIONS: Men had markedly higher serum cTnI compared with women, although they were case matched with respect to age and cardiac risk factors. Our results may suggest there may be sex-related differences in the myocardial response to ischemia and reperfusion injury or intrinsic differences between the male and female myocardium.