Judith Therrien

Pulmonary valve replacement in adults late after repair of tetralogy of fallot : Are We operating too late?

OBJECTIVES The purpose of this study is to evaluate right ventricular (RV) volume and function after pulmonary valve replacement (PVR) and to address the issue of optimal surgical timing in these patients. BACKGROUND Chronic pulmonary regurgitation (PR) following repair of tetralogy of Fallot (TOF) leads to RV dilation and an increased incidence of sudden cardiac death in adult patients. METHODS We studied 25 consecutive adult patients who underwent PVR for significant PR late after repair of TOF. Radionuclide angiography was performed in all at a mean of 8.2 months (+/- 8 months) before PVR and repeated at a mean of 28.0 months (+/- 22.8 months) after the operation. Right ventricular (RV) end-systolic volume (RVESV), RV end-diastolic volume (RVEDV) and RV ejection fraction (RVEF) were measured. RESULTS Mean RVEDV, RVESV and RVEF remained unchanged after PVR (227.1 ml versus 214.9 ml, p = 0.74; 157.4 ml versus 155.4 ml, p = 0.94; 35.6% versus 34.7%, p = 0.78, respectively). Of the 10 patients with RVEF > or = 0.40 before PVR, 5 patients (50%) maintained a RVEF > or = 0.40 following PVR, whereas only 2 out of 15 patients (13%) with pre-operative values <0.40 reached an RVEF > or = 0.40 postoperatively (p < 0.001). CONCLUSIONS Right ventricular recovery following PVR for chronic significant pulmonary regurgitation after repair of TOF may be compromised in the adult population. In order to maintain adequate RV contractility, pulmonary valve implant in these patients should be considered before RV function deteriorates.

Atrial Arrhythmias in Adults With Congenital Heart Disease

Background— Atrial arrhythmias increase disease burden in the general adult population. Adults with congenital heart lesions constitute a rapidly growing group of patients with cardiovascular disease. We hypothesized that atrial arrhythmias increase with age and impair health outcomes in this population. Methods and Results— We conducted a population-based analysis of prevalence, lifetime risk, mortality, and morbidity associated with atrial arrhythmias in adults with congenital heart disease from l983 to 2005. In 38 428 adults with congenital heart disease in 2005, 5812 had atrial arrhythmias. Overall, the 20-year risk of developing atrial arrhythmia was 7% in a 20-year-old subject and 38% in a 50-year-old subject. More than 50% of patients with severe congenital heart disease reaching age 18 years developed atrial arrhythmias by age 65 years. In patients with congenital heart disease, the hazard ratio of any adverse event in those with atrial arrhythmias compared with those without was 2.50 (95% confidence interval, 2.38 to 2.62; P<0.0001), with a near 50% increase in mortality (hazard ratio, 1.47; 95% confidence interval, 1.37 to 1.58; P<0.001), more than double the risk of morbidity (stroke or heart failure) (hazard ratio, 2.21; 95% confidence interval, 2.07 to 2.36; P<0.001), and 3 times the risk of cardiac interventions (hazard ratio, 3.00; 95% confidence interval, 2.81 to 3.20; P<0.001). Conclusions— Atrial arrhythmias occurred in 15% of adults with congenital heart disease. The lifetime incidence increased steadily with age and was associated with a doubling of the risk of adverse events. An increase in resource allocation should be anticipated to deal with this increasing burden.

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Outflow tract obstruction, coarctation of the aorta, tetralogy of Fallot, Ebstein anomaly and Marfan’s syndrome

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part II of the guidelines includes recommendations for the care of patients with left ventricular outflow tract obstruction and bicuspid aortic valve disease, coarctation of the aorta, right ventricular outflow tract obstruction, tetralogy of Fallot, Ebstein anomaly and Marfans syndrome. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts that are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.

Assessment of Systemic Right Ventricular Function in Patients With Transposition of the Great Arteries Using the Myocardial Performance Index Comparison With Cardiac Magnetic Resonance Imaging

Background—Assessment of systemic right ventricular (RV) function is a key point in the follow-up of patients with transposition of the great arteries (TGA). Current echocardiographic assessment of RV function is at best an estimate, and cardiac magnetic resonance (CMR) is considered the gold standard. However, this technique is expensive, has limited availability, and requires significant expertise to acquire and interpret the images. The myocardial performance index (MPI) has recently been studied for assessment of pulmonary RV function and shows promise as a simple yet powerful tool for assessing patients with RV dysfunction of various origins. We set out to compare MPI and CMR assessment of systemic RV function in patients with TGA. Methods and Results—Data from patients with TGA (11 with congenitally corrected TGA, 18 with surgically corrected TGA) who had CMR within 6 months of their echocardiogram were reviewed. The average systemic RV ejection fraction (RVEF) by CMR was 39.4±11.4%, and the systemic RVMPI for this group was 0.56±0.21. There was a strong negative correlation between the systemic RVMPI and systemic RVEF by CMR (r=−0.82, P<0.01). The systemic RVEF can be estimated from this formula: RVEF=65%−(45.2×MPI). Conclusions—MPI can be used in patients with systemic RVs to assess global function and to estimate an EF with good accuracy.

Angiotensin-Converting Enzyme Inhibitors in Adults After the Mustard Procedure

Angiotensin-converting enzyme inhibitors had no significant effect on cardiopulmonary exercise function in 14 patients who had undergone a Mustard operation for transposition of the great arteries. In some patients aerobic capacity improved and maximum systolic blood pressure decreased.

Specialized Adult Congenital Heart Disease Care The Impact of Policy on Mortality

Background— Clinical guidelines recommend specialized care for adult congenital heart disease (ACHD) patients. In reality, few patients receive such dedicated care. We sought to examine the impact of specialized care on ACHD patient mortality. Methods and Results— We examined referral rates to specialized ACHD centers and ACHD patient mortality rates between 1990 and 2005 in the population-based Quebec Congenital Heart Disease database (n=71 467). This period covers several years before and after the publication of guidelines endorsing specialized care for ACHD patients. A time-series design, based on Joinpoint and Poisson regression analyses, was used to assess the changes in annual referral and patient mortality rates. The association between specialized ACHD care and all-cause mortality was assessed in both case–control and cohort studies. The time-series analysis demonstrated a significant increase in referral rates to specialized ACHD centers in 1997 (rate ratio, +7.4%; 95% confidence interval [CI], +6.6% to +8.2%). In parallel, a significant reduction in expected ACHD patient mortality was observed after year 2000 (rate ratio, −5.0%;95% CI, −10.8% to −0.8%). In exploratory post hoc cohort and case–control analyses, specialized ACHD care was independently associated with reduced mortality (hazard ratio, 0.78; 95% CI, 0.65–0.94) and a reduced odds of death (adjusted odds ratio, 0.82; 95% CI, 0.08–0.97), respectively. This effect was predominantly driven by patients with severe congenital heart disease (hazard ratio, 0.38; 95% CI, 0.22–0.67). Conclusions— A significant increase in referrals to specialized ACHD centers followed the introduction of the clinical guidelines. Moreover, referral to specialized ACHD care was independently associated with a significant mortality reduction. Our findings support a model of specialized care for all ACHD patients.Background— Clinical guidelines recommend specialized care for adult congenital heart disease (ACHD) patients. In reality, few patients receive such dedicated care. We sought to examine the impact of specialized care on ACHD patient mortality. Methods and Results— We examined referral rates to specialized ACHD centers and ACHD patient mortality rates between 1990 and 2005 in the population-based Quebec Congenital Heart Disease database (n=71 467). This period covers several years before and after the publication of guidelines endorsing specialized care for ACHD patients. A time-series design, based on Joinpoint and Poisson regression analyses, was used to assess the changes in annual referral and patient mortality rates. The association between specialized ACHD care and all-cause mortality was assessed in both case–control and cohort studies. The time-series analysis demonstrated a significant increase in referral rates to specialized ACHD centers in 1997 (rate ratio, +7.4%; 95% confidence interval [CI], +6.6% to +8.2%). In parallel, a significant reduction in expected ACHD patient mortality was observed after year 2000 (rate ratio, −5.0%;95% CI, −10.8% to −0.8%). In exploratory post hoc cohort and case–control analyses, specialized ACHD care was independently associated with reduced mortality (hazard ratio, 0.78; 95% CI, 0.65–0.94) and a reduced odds of death (adjusted odds ratio, 0.82; 95% CI, 0.08–0.97), respectively. This effect was predominantly driven by patients with severe congenital heart disease (hazard ratio, 0.38; 95% CI, 0.22–0.67). Conclusions— A significant increase in referrals to specialized ACHD centers followed the introduction of the clinical guidelines. Moreover, referral to specialized ACHD care was independently associated with a significant mortality reduction. Our findings support a model of specialized care for all ACHD patients. # CLINICAL PERSPECTIVE {#article-title-51}

Geriatric Congenital Heart Disease: Burden of Disease and Predictors of Mortality

OBJECTIVES The study sought to measure the prevalence, disease burden, and determinants of mortality in geriatric adults with congenital heart disease (ACHD). BACKGROUND The population of ACHD is increasing and aging. The geriatric ACHD population has yet to be characterized. METHODS Population-based cohort study using the Quebec Congenital Heart Disease Database of all patients with congenital heart disease coming into contact with the Quebec healthcare system between 1983 and 2005. Subjects with specific diagnoses of congenital heart disease and age 65 years at time of entry into the cohort were followed for up to 15 years. The primary outcome was all-cause mortality. RESULTS The geriatric ACHD cohort consisted of 3,239 patients. From 1990 to 2005, the prevalence of ACHD in older adults remained constant from 3.8 to 3.7 per 1,000 indexed to the general population (prevalence odds ratio: 0.98; 95% confidence interval [CI]: 0.93 to 1.03). The age-stratified population prevalence of ACHD was similar in older and younger adults. The most common types of congenital heart disease lesions in older adults were shunt lesions (60%), followed by valvular lesions (37%) and severe congenital heart lesions (3%). Type of ACHD and ACHD-related complications had a minor impact on mortality, which was predominantly driven by acquired comorbid conditions. The most powerful predictors of mortality in the Cox proportional hazards model were: dementia (hazard ratio [HR]: 3.24; 95% CI: 1.53 to 6.85), gastrointestinal bleed (HR: 2.79; 95% CI: 1.66 to 4.69), and chronic kidney disease (HR: 2.50; 95% CI: 1.72 to 3.65). CONCLUSIONS The prevalence of geriatric ACHD is substantial, although severe lesions remain uncommon. ACHD patients that live long enough acquire general medical comorbidities, which are the pre-eminent determinants of their mortality.

Diagnosis of Pulmonary Hypertension in the Congenital Heart Disease Adult Population: Impact on Outcomes

OBJECTIVES The aim of this study was to assess the impact of the diagnosis of pulmonary hypertension (PH) on mortality, morbidity, and health services utilization (HSU) in an adult congenital heart disease (CHD) population. BACKGROUND Although PH is a well-recognized complication of CHD, population-based studies of its significance on the survival and functional capacity of patients are uncommon. METHODS A retrospective longitudinal cohort study was conducted in an adult CHD population with 23 years of follow-up, from 1983 to 2005. The prevalence of PH was measured in 2005. Mortality, morbidity, and HSU outcomes were compared between patients with and without diagnoses of PH using multivariate Cox (mortality and morbidity) and Poisson (HSU) regression models within a subcohort matched for age and CHD lesion type. RESULTS Of 38,430 adults alive with CHD in 2005, 2,212 (5.8%) had diagnoses of PH (median age 67 years, 59% women). The diagnosis of PH increased the all-cause mortality rate of adults with CHD more than 2-fold compared with patients without PH (hazard ratio [HR]: 2.69; 95% confidence interval [CI]: 2.41 to 2.99). Morbid complications including heart failure and arrhythmia occurred with a 3-fold higher risk compared with patients without PH (HR: 3.01; 95% CI: 2.80 to 3.22). The utilization of inpatient and outpatient services was increased, especially cardiac catheterization, excluding the index diagnostic study (rate ratio: 5.04; 95% CI: 4.27 to 5.93) and coronary and intensive care hospitalizations (rate ratio: 5.03; 95% CI: 4.86 to 5.20). CONCLUSIONS A diagnosis of PH in adults with CHD is associated with a more than 2-fold higher risk for all-cause mortality and 3-fold higher rates of HSU, reflecting high morbidity.

Pressure Half-time Predicts Hemodynamically Significant Pulmonary Regurgitation in Adult Patients with Repaired Tetralogy of Fallot

Pulmonary regurgitation (PR) is associated with adverse outcomes late after tetralogy of Fallot repair. Accurate assessment of PR in these patients is, therefore, fundamental to their clinical treatment; however, accurate ultrasound markers of severity are as yet poorly defined. This is a prospective study of 34 adult patients with repaired tetralogy of Fallot. Cardiac magnetic resonance imaging was used to assess the PR fraction and its hemodynamic significance on the right ventricular volumes. Regurgitant fractions >/= 20% were associated with significant increases in right ventricular end-diastolic volumes. Echocardiographic continuous wave Doppler profiles of the PR jet were used to calculate pressure half-time. Pulmonary pressure half-time < 100 milliseconds was found to be a good indicator of hemodynamically significant regurgitation. This measure is highly reproducible and easily accessible.

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Complex congenital cardiac lesions

With advances in pediatric cardiology and cardiac surgery, the population of adults with congenital heart disease (CHD) has increased. In the current era, there are more adults with CHD than children. This population has many unique issues and needs. They have distinctive forms of heart failure and their cardiac disease can be associated with pulmonary hypertension, thromboemboli, complex arrhythmias and sudden death. Medical aspects that need to be considered relate to the long-term and multisystemic effects of single ventricle physiology, cyanosis, systemic right ventricles, complex intracardiac baffles and failing subpulmonary right ventricles. Since the 2001 Canadian Cardiovascular Society Consensus Conference report on the management of adults with CHD, there have been significant advances in the field of adult CHD. Therefore, new clinical guidelines have been written by Canadian adult CHD physicians in collaboration with an international panel of experts in the field. Part III of the guidelines includes recommendations for the care of patients with complete transposition of the great arteries, congenitally corrected transposition of the great arteries, Fontan operations and single ventricles, Eisenmengers syndrome, and cyanotic heart disease. Topics addressed include genetics, clinical outcomes, recommended diagnostic workup, surgical and interventional options, treatment of arrhythmias, assessment of pregnancy risk and follow-up requirements. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology. The complete document and references can also be found at www.ccs.ca or www.cachnet.org.