Ruptured Sinus of Valsalva Aneurysm Associated with Situs Ambiguus, Isolated Levocardia, and Polysplenia.

Aneurysm of the sinus of Valsalva, a rare cardiac condition, results from dilation of an aortic sinus. Sudden aneurysm rupture can trigger rapidly progressive heart failure. We discuss the case of a 57-year-old woman with situs ambiguus, isolated levocardia, and polysplenia who presented with acute-onset heart failure. Transesophageal echocardiograms revealed an aneurysm of the right coronary sinus of Valsalva that had ruptured into the right atrial cavity. The patient underwent successful surgical repair. To our knowledge, this is the first report of a sinus of Valsalva aneurysm in a patient with this combination of congenital abnormalities. We briefly review the association between congenital heart disease, situs ambiguus, and ciliary dysfunction.

Determining the best ventilatory efficiency measure to predict mortality in patients with heart failure.

BACKGROUND: Ventilatory efficiency, the relationship between ventilation (VE) and carbon dioxide production (VCO2), predicts mortality in heart failure patients, but its determination has not been standardized. Additionally, it is unclear if the prognostic power of ventilatory efficiency is independent of exercise intensity. Therefore, we investigated the relative prognostic power of different measures of ventilatory efficiency calculated from maximal and sub-maximal exercise in patients with heart failure. METHODS AND RESULTS: Heart failure patients (n = 355, 72% males, age 51 +/- 10 years) had follow-up for at least 5 years from an exercise test. There were 145 events (133 deaths and 12 emergent cardiac transplants). Ventilatory efficiency calculations were not equivalent. Of the different measures of ventilatory efficiency, the VE/VCO2 slope to peak exercise was the most significant predictor of mortality in a multivariable Cox model, including ejection fraction, systolic blood pressure, peak oxygen consumption (VO2), gender, etiology, and heart rate. A 5 U increment of the VE/VCO2 slope to peak exercise corresponded to a 9% increase in mortality risk. When tests were grouped by peak exercise intensity, by quartiles of peak respiratory exchange ratio, the VE/VCO2 slope to peak exercise was always a better predictor than peak VO2. CONCLUSION: Peak and sub-maximal measures of ventilatory efficiency were not equivalent, and the VE/VCO2 slope to peak exercise was the best predictor of mortality in patients with heart failure. Thus, the prognostic power of ventilatory efficiency is enhanced when exercise extends beyond the ventilatory threshold and includes all of the available exercise data.