Huge aneurysm of the ascending aorta in a patient with adult-type Pompe's disease: histological findings mimicking fibrillinopathy.

Adult-type Pompe's disease (glycogen storage disease type II) has rarely been shown to present with dilatative arteriopathy, suggesting potential smooth muscle involvement in addition to lysosomal glycogen deposits usually restricted to skeletal muscle tissue. We report the case of a middle-aged man under enzyme replacement therapy presenting with an exceedingly large thoracic aortic aneurysm. Surprisingly, the histological work-up of resected aortic tissue revealed changes mimicking those observed in patients with classic connective tissue diseases. Enzyme replacement therapy, in addition to musculoskeletal and pulmonary treatment for patients with Pompe's disease, may prolong survival and lead to patients presenting with vascular alterations that may pose surgical and potential diagnostic challenges in the future.

Comparison of arterial and venous coronary artery bypass flow measurements using 3-T magnetic resonance phase contrast imaging.

OBJECTIVE: Comparison of arterial and venous coronary artery bypass flow measurements using 3-T magnetic resonance (MR) phase contrast in correlation with intraoperative Doppler flow measurements. METHODS: Fifty-six coronary bypasses (right coronary artery n=18, left internal mammary artery to left anterior descending artery n=16, marginal artery n=7, circumflex artery n=7, diagonal artery n=6, left anterior descending artery n=1, and right internal mammary artery to right coronary artery n=1) were studied in 27 asymptomatic patients. In this prospective study, each bypass was studied intra-operatively using Doppler flow measurement. Within one week post surgery, patients were studied using a 3-T MR scanner (Magnetom Verio, Siemens, Erlangen, Germany) using velocity encoded phase-contrast flow measurements. RESULTS: Intraoperative Doppler flow measurements demonstrated regular flow patterns in all vascular territories supplied. All bypasses were patent on MRI and flow measurement results were as follows: median flow 60ml/min (interquartile range (IQR): 37.5-78.5ml/min). For comparison, the corresponding median intraoperative flow was 58ml/min (IQR: 41-80ml/min) (p<0.001; R=0.44). Linear regression analysis demonstrated a significant correlation for venous bypasses (p=0.0002; R=0.48), but not for arterial bypasses (p=0.09; R=0.24). CONCLUSION: This study demonstrated that MR flow measurements of venous bypass grafts agreed more with Doppler than arterial bypass grafts. However, bypass patency was confirmed for all patients. In the future, this technique may be used for non invasive coronary bypass graft follow-up.

Computed tomographic coronary angiography in patients with surgically treated type A aortic dissection: preliminary results.

Acute type A aortic dissection is a serious emergency with a mortality rate of up to 40% within the first 24 h when left untreated. Surgical therapy needs to be initiated promptly. Due to this urgent situation, preoperative evaluation of the coronary arteries is not routinely performed in these patients. The aim of this study was to evaluate the accuracy of 64-slice computed tomography angiography (CTA) for postoperative coronary artery assessment in these patients. Ten consecutive patients with two or more cardiovascular risk factors were prospectively enrolled. Patients had type A aortic dissection treated surgically with a supracoronary graft of the ascending aorta. Performance of CTA to exclude significant stenosis (>50% lumen narrowing) and/or coronary artery dissection was compared with quantitative coronary angiography. A total of 147 segments were evaluated. Three segments (2%) were excluded from analysis. CTA correctly assessed one of three significant stenoses in three patients and correctly excluded coronary artery disease (CAD) in six of ten patients. One patient was rated false positive. Overall accuracy, sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) of CT for identifying coronary artery disease by segment was 98%, 33%, 99%, 50%, and 99%, respectively (P<0.05). By patient, it was 70%, 33%, 86%, 50%, and 75%, respectively. No coronary artery dissection was found. Noninvasive CTA may be a viable alternative to conventional angiography for postoperative coronary artery evaluation in patients with surgically treated type A aortic dissection and cardiovascular risk factors. An NPV of 99% should allow for reliable exclusion of CAD. Further studies with higher patient numbers are warranted.

Benefits of cardiac surgery in octogenarians--a postoperative quality of life assessment.

OBJECTIVE: Assessment of postoperative quality of life in patients over 80 years after cardiac surgery including coronary artery bypass grafting (CABG), aortic valve replacement (AVR) and combined procedures. METHODS: Quality of life of n=136 patients over 80 years at operation (82.3+/-2.1 years), undergoing isolated CABG in 61 patients (45%), isolated AVR in 34 patients (25%) and a combination of CABG and AVR in 41 patients (30%) between January 1999 and December 2003 was reviewed. Preoperatively 66.2% presented in NYHA-class III/IV or CCS-class III/IV. Mean ejection fraction (EF) was 59.5%+/-14.0 (range 25-90%). Quality of life assessment was performed via a Seattle Angina Questionnaire. Follow-up was 100% complete for a total of 890 days (69-1853 days). RESULTS: Five-year survival was 70% for the CABG group, 75% for the AVR group and 65% for the CABG/AVR group. Quality of life was remarkable in all of the three groups after surgery. Overall 97 patients (81%) were not or little disabled in their daily activity. One hundred and twelve patients (93%) were free or considerably less symptomatic. Seventy-eight patients or 65% reported to be very satisfied with their current quality of life and 112 patients (93%) felt very reassured to have continuous full access to medical treatment despite of their advanced age. CONCLUSIONS: A remarkable quality of life and important improvement in the functional status after cardiac surgery in patients over 80 paired with a satisfactory medium-term survival justify early intervention for heart disease in this age group. Therefore, referral practice for patients over 80 years for heart surgery should be handled liberally.