Leeanne Grigg

The Fontan Procedure Contemporary Techniques Have Improved Long-Term Outcomes

Background— To determine whether patients undergoing the lateral tunnel and extracardiac conduit modifications of the Fontan procedure have better outcomes than patients undergoing a classical atriopulmonary connection. Methods and Results— Between 1980 and 2000, 305 consecutive patients underwent a Fontan procedure at our institution. There were 10 hospital deaths (mortality: 3%) with no death after 1990. Independent risk factors for mortality were preoperative elevated pulmonary artery pressures (P=0.002) and common atrioventricular valve (P=0.04). Fontan was taken down during hospital stay in 7 patients. A mean of 12±6 years of follow-up was obtained in the 257 nonforeign Fontan survivors. Completeness of concurrent follow-up was 96%. Twenty-year survival was 84% (95% CI: 79 to 89%). Recent techniques improved late survival. The 15-year survival after atriopulmonary connection was 81% (95% CI: 73% to 87%) versus 94% (95% CI: 79% to 98%) for lateral tunnel (P=0.004). Nine pts required heart transplantation (8 atriopulmonary connection, 1 lateral tunnel). Undergoing a Fontan modification independently predicted decreased occurrence of arrhythmia, and 15-year freedom from SVT was 61% (95% CI: 51% to 70%) for atriopulmonary connection versus 87% (95% CI: 76% to 93%) for lateral tunnel (P=0.02). Freedom from Fontan failure (death, take-down, transplantation, or NYHA class III-IV) was 70% (95% CI: 58% to 79%) at 20 years. After extra-cardiac conduits, no death, SVT, or failure was observed. Conclusions— The Fontan procedure remains a palliation, but outcomes of patients have improved. Better patient selection minimizes hospital mortality. Patients with lateral tunnel and extracardiac conduit modifications experience less arrhythmia and are likely to have failure of their Fontan circulation postponed.

Redefining Expectations of Long-Term Survival After the Fontan Procedure Twenty-Five Years of Follow-Up From the Entire Population of Australia and New Zealand

Background— The life expectancy of patients undergoing a Fontan procedure is unknown. Methods and Results— Follow-up of all 1006 survivors of the 1089 patients who underwent a Fontan procedure in Australia and New Zealand was obtained from a binational population-based registry including all pediatric and adult cardiac centers. There were 203 atriopulmonary connections (AP; 1975–1995), 271 lateral tunnels (1988–2006), and 532 extracardiac conduits (1997–2010). The proportion with hypoplastic left heart syndrome increased from 1/173 (1%) before 1990 to 80/500 (16%) after 2000. Survival at 10 years was 89% (84%–93%) for AP and 97% (95% confidence interval [CI], 94%–99%) for lateral tunnels and extracardiac conduits. The longest survival estimate was 76% (95% CI, 67%–82%) at 25 years for AP. AP independently predicted worse survival compared with extracardiac conduits (hazard ratio, 6.2; P<0.001; 95% CI, 2.4–16.0). Freedom from failure (death, transplantation, takedown, conversion to extracardiac conduits, New York Heart Association III/IV, or protein-losing enteropathy/plastic bronchitis) 20 years after Fontan was 70% (95% CI, 63%–76%). Hypoplastic left heart syndrome was the primary predictor of Fontan failure (hazard ratio, 3.8; P<0.001; 95% CI, 2.0–7.1). Ten-year freedom from failure was 79% (95% CI, 61%–89%) for hypoplastic left heart syndrome versus 92% (95% CI, 87%–95%) for other morphologies. Conclusions— The long-term survival of the Australia and New Zealand Fontan population is excellent. Patients with an AP Fontan experience survival of 76% at 25 years. Technical modifications have further improved survival. Patients with hypoplastic left heart syndrome are at higher risk of failure. Large, comprehensive registries such as this will further improve our understanding of late outcomes after the Fontan procedure.

Transesophageal Doppler echocardiography in obstructive hypertrophic cardiomyopathy: clarification of pathophysiology and importance in intraoperative decision making.

To better understand the pathophysiology of obstruction of left ventricular outflow in hypertrophic cardiomyopathy and to determine the value of intraoperative transesophageal Doppler echocardiography in decision making, 32 consecutive patients undergoing ventriculomyectomy were assessed. The mean preoperative left ventricular outflow gradient was 83 +/- 39 mm Hg and the mean basal septal width was 24 +/- 6 mm. Compared with transesophageal findings in 10 normal control subjects, the mitral leaflets were longer and the coaptation point was abnormal in the patients with obstructive hypertrophic cardiomyopathy (anterior and posterior leaflet lengths in the patients were 31 +/- 4 vs. 22 +/- 3 mm in the control group [p less than 0.00001] and 20 +/- 2 vs. 15 +/- 3 mm in the control group [p less than 0.00001]). The coaptation point in the patient group was in the body of the leaflets at a mean of 9 +/- 2 mm from the anterior leaflet tip, whereas it was at or within 3 mm of the leaflet tip in the normal group. During early systole, the distal third to half of the anterior mitral leaflet angled sharply anteriorly and superiorly (systolic anterior motion), resulting in leaflet-septal contact and incomplete mitral leaflet coaptation in mid-systole. This caused the formation of a funnel, composed of the distal parts of both leaflets, that allowed a jet of posteriorly directed mitral regurgitation to occur in mid- and late systole. The sequence of events in systole was eject/obstruct/leak. Transesophageal echocardiography was also helpful in planning the extent of the resection, assessing the immediate result and excluding important complications. In successful cases, the post-myectomy study showed 1) a dramatic thinning of the septum, with widening of the left ventricular outflow tract to a width similar to that in the normal subjects, 2) resolution of systolic anterior motion and the left ventricular outflow tract color mosaic, and marked reduction or abolition of mitral regurgitation despite persistence of abnormal mitral leaflet length and an abnormal mitral leaflet coaptation point. The routine use of transesophageal echocardiography in patients undergoing surgical myectomy for the treatment of obstructive hypertrophic cardiomyopathy is recommended.

Determinants of restenosis and lack of effect of dietary supplementation with eicosapentaenoic acid on the incidence of coronary artery restenosis after angioplasty

The effect of an eicosapentaenoic acid-rich encapsulated preparation of fish oil on the incidence of early restenosis after coronary angioplasty was assessed by a randomized double-blind placebo-controlled study. A total of 108 patients received either 10 capsules of fish oil (1.8 g eicosapentaenoic acid, 1.2 g docosahexaenoic acid) or 10 control capsules (50% olive oil, 50% corn oil), commencing the day before angioplasty and continuing for 4 months after angioplasty, in addition to treatment with aspirin and verapamil. In 101 (94%) of the 108 patients, follow-up angiographic or postmortem result was evaluated at a mean (+/- SD) of 100 (+/- 22) days. Angiographic restenosis was observed in 34% of patients (29% of lesions) in the fish oil-treated group and 33% of patients (31% of lesions) in the control group (no significant difference). The overall incidence of angiographic restenosis was significantly higher in patients with 1) recurrent angina pectoris, 2) a positive exercise test at follow-up after angioplasty, 3) residual stenosis greater than 30% immediately after angioplasty, and 4) dilation of the left anterior descending or right coronary artery. Biochemical investigations showed a greater decrease in the serum triglyceride levels in the fish oil-treated group versus the control group (p less than 0.05) but no differences between the two groups in cholesterol levels or platelet counts over the 4 month period. In conclusion, in this study, the administration of fish oil at a dose of 10 capsules/day did not reduce the incidence of early restenosis after coronary angioplasty.

Effect of Chronic Right Atrial Stretch on Atrial Electrical Remodeling in Patients With an Atrial Septal Defect

Background—Adults with an atrial septal defect (ASD) frequently develop late atrial arrhythmias. We sought to characterize the pattern and persistence of atrial electrical remodeling caused by chronic right atrial (RA) stretch in this group. Methods and Results—Thirteen ASD patients without atrial arrhythmia (42±10 years old; RA volume, 65±16 mL) and 17 normal control subjects (44±11 years old; RA volume, 38±8 mL) had electrophysiological study to measure (1) atrial effective refractory period (AERP) from the low lateral/high lateral/high septal RA and distal coronary sinus (CS), (2) dispersion of AERP, (3) lateral-RA and CS conduction time during constant pacing, (4) conduction delay across the crista terminalis measuring the number of crista catheter bipoles (0–10) recording discrete double potentials during pacing, (5) corrected sinus node recovery time, and (6) P-wave duration. After ASD closure (8.3±5.6 months), follow-up echo studies (n=12) and electrophysiological study (n=4) were performed. The low-lateral AERP, P-wave duration, sinus node recovery time, and extent of conduction delay across the crista terminalis were significantly greater in ASD patients. No differences were found for other measured electrophysiological study parameters. At follow-up, there was incomplete resolution of RA volume (47±12 mL;P <0.01 versus before surgery), a trend toward shortening of the AERP at the lateral RA and an increase at the distal CS and high septal RA, but persisting extensive, widely split crista double potentials. Conclusions—Chronic RA stretch because of ASD causes electrical remodeling with modest increases in RA ERP, conduction delay at the crista terminalis, and sinus node dysfunction. Conduction delay at the crista terminalis persists beyond ASD closure and may contribute to the long-term atrial arrhythmia substrate in this condition.

The porous titanium steroid eluting electrode:a double blind study assessing the stimulation threshold effects of steroid.

A transvenous pacing lead with a porous electrode which slowly elutes the steroid, dexamethasone sodium phosphate, has been developed. Previous investigations show low and constant stimulation thresholds persisting over at least the first two years post‐implantation. As it is not known whether this low threshold results from the steroid or electrode configuration, a double blind study was designed to compare the same electrode configuration with and without steroid over a 2‐year follow‐up period. There were ten patients in each group with similar age, sex, indications for pacing and implantation data. Regular measurements of postoperative pulse duration thresholds were performed using a customized VVIM pulse generator programmed to 1.5 V output. For the first two days post‐implantation, there were no statistical differences in the pulse duration thresholds between the two pacing leads. From 2 weeks to 2 years the pulse duration thresholds for the steroid leads remained almost constant, whereas the leads without steroid showed a typical rise. The difference in pulse duration thresholds between the two groups of leads from two weeks onwards confirmed that it was the steroid rather than the electrode configuration which prevented the rise in chronic stimulation threshold.

Outcomes of the Arterial Switch Operation for Transposition of the Great Arteries: 25 Years of Experience

BACKGROUND Studies on long-term outcomes of the arterial switch operation (ASO) for transposition of the great arteries (TGA) are uncommon. Thus, we sought to determine the long-term outcomes for patients after ASO performed at a single institution over a 25-year period. METHODS From 1983 to 2009, 618 patients underwent the ASO for TGA and were reviewed retrospectively. RESULTS Overall early mortality was 2.8%. Risk factors for early death on multivariate analysis were resection of left ventricular outflow tract obstruction at time of ASO (p = 0.001), weight less than 2.5 kg at time of ASO (p < 0.001), associated aortic arch obstruction (p = 0.043), and the need for postoperative extracorporeal membrane oxygenation (p < 0.001). Mean follow-up time was 10.6 years (range 2 months to 26.1 years). Late mortality was 0.9%. Reintervention was significantly higher (p < 0.001) in patients with ventricular septal defect or arch obstruction versus those without them (25.2% and 23.4% vs 5.9% at 15- year follow-up). Risk factors for late reintervention were left ventricular outflow tract obstruction at time of ASO (p < 0.001) and a greater circulatory arrest time (p < 0.001). Freedom from at least moderate neoaortic valve regurgitation for the entire cohort was 98.7% (95% confidence interval 96.8 to 99.5%) at 20 years. Mild neoaortic regurgitation was seen in 25.6% of patients at mean follow-up. All patients were free of arrhythmia and heart failure symptoms at last follow-up. CONCLUSIONS The ASO can be performed with good long-term results. Patients with associated ventricular septal defect and aortic arch obstruction warrant close follow-up.

Predictors of survival after single-ventricle palliation: the impact of right ventricular dominance.

OBJECTIVES This study examined survival after surgical palliation in children with single-ventricle physiology. BACKGROUND Contemporary surgical outcomes for the entire population of newborns undergoing single-ventricle palliation are unclear. METHODS In a single-center review of 499 consecutive patients undergoing univentricular palliation from 1990 to 2008, predictors of mortality were determined using multivariate risk analysis, stratified for each post-operative stay and interim states. RESULTS After 2000, the population comprised more patients with dominant right ventricle (66% vs. 36%) and hypoplastic left heart syndrome (HLHS) (47% vs. 13%). Median age at bidirectional cavopulmonary shunt (BCPS) decreased from 15 months (10 to 22 months) before 2000 to 4 months (3.3 to 9 months) thereafter. Survival rates at 1, 5, and 10 years were, respectively, 82% (95% confidence interval [CI]: 79% to 85%), 74% (95% CI: 70% to 78%), and 71% (95% CI: 67% to 75%). Throughout the study, atrioventricular valve regurgitation (hazard ratio [HR]: 1.8; p = 0.008), not having transposition (HR: 2.0; p = 0.013), and heterotaxia (HR: 2.0; p = 0.026) were predictors of mortality. The most potent risk factor was right ventricular (RV) dominance (HR: 2.2; p = 0.001) because of its impact before BCPS. HR for death in patients with RV dominance went from 2.8 (95% CI: 1.4 to 5.7; p = 0.005) before BCPS to 1.0 (95% CI: 0.5 to 2.1; p = 0.98) thereafter. Survival of patients with RV dominance, adjusted for the risk factors noted here, improved over the study period (p = 0.001). CONCLUSIONS Considerable mortality is still observed during the first years of life among patients with single ventricle. RV dominance is the most important risk factor for death but only before BCPS.

Attendance at cardiac rehabilitation is associated with lower all-cause mortality after 14 years of follow-up

Objective To investigate whether attendance at cardiac rehabilitation (CR) independently predicts all-cause mortality over 14 years and whether there is a dose–response relationship between the proportion of CR sessions attended and long-term mortality. Design Retrospective cohort study. Setting CR programmes in Victoria, Australia Patients The sample comprised 544 men and women eligible for CR following myocardial infarction, coronary artery bypass surgery or percutaneous interventions. Participants were tracked 4 months after hospital discharge to ascertain CR attendance status. Main outcome measures All-cause mortality at 14 years ascertained through linkage to the Australian National Death Index. Results In total, 281 (52%) men and women attended at least one CR session. There were few significant differences between non-attenders and attenders. After adjustment for age, sex, diagnosis, employment, diabetes and family history, the mortality risk for non-attenders was 58% greater than for attenders (HR=1.58, 95% CI 1.16 to 2.15). Participants who attended <25% of sessions had a mortality risk more than twice that of participants attending ≥75% of sessions (OR=2.57, 95% CI 1.04 to 6.38). This association was attenuated after adjusting for current smoking (OR=2.06, 95% CI 0.80 to 5.29). Conclusions This study provides further evidence for the long-term benefits of CR in a contemporary, heterogeneous population. While a dose–response relationship may exist between the number of sessions attended and long-term mortality, this relationship does not occur independently of smoking differences. CR practitioners should encourage smokers to attend CR and provide support for smoking cessation.

Transcranial Doppler Detection of Microemboli During Percutaneous Transluminal Coronary Angioplasty

BACKGROUND AND PURPOSE The use of percutaneous transluminal coronary angioplasty (PTCA) to treat coronary artery disease is now commonplace. The occurrence of microemboli during invasive procedures such as cardiac angiography and bypass surgery is well documented, although neurological complications are relatively uncommon. To date, no investigation has been undertaken of the frequency or nature of microemboli occurring during PTCA or of the correlation with aortic atheroma. METHODS Twenty patients having elective PTCA underwent examination by transcranial Doppler ultrasonography (TCD) to detect left middle cerebral artery microemboli occurring during the procedure. Blinded off-line analysis correlated microembolic signal counts on TCD with the components of each stage of the PTCA. Patients later underwent transesophageal (TEE) echocardiography, with measurements made of the thickness of the intima and atheroma in the ascending and descending thoracic aortic arch by cardiologists blinded to the TCD results. RESULTS A total of 973 microembolic signals were detected (mean+/-SD, 48.7+/-36.7 per patient); 196 (20%) occurred on movement of the PTCA catheter and wire around the aortic arch, 84 (9%) with other PTCA catheter-associated movements, and 679 (70%) in association with injection of solutions (eg, saline and contrast). Mean signal counts during contrast injection were significantly greater than during the other 3 phases (P<0.001). No neurological events occurred in the study. Although not statistically significant, there was a trend toward greater microembolic signal counts with the number of times the catheter was passed around the aortic arch and the amount of arch atheroma detected by transesophageal echocardiography. CONCLUSIONS Microemboli detected on TCD are a common occurrence during PTCA but are largely asymptomatic. The majority of microembolic signals are most probably gaseous in origin and do not appear to be related to the extent of aortic atheroma or to clinical events.