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

Abstract

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, Eisenmenger's 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.

Canadian Cardiovascular Society 2009 Consensus Conference on the management of adults with congenital heart disease: Executive summary

“…The present executive summary is an abbreviated version of the consensus conference, focusing on the recommendations pertaining to medical therapy and interventions. The complete document consists of four manuscripts, which are published online in the present issue of The Canadian Journal of Cardiology (3)(4)(5)(6), and includes more detailed recommendations including sections on genetics, clinical outcomes, suggested diagnostic workup, surgical and interventional options and outcomes, treatment of arrhythmias, assessment of pregnancy and follow-up requirements. The complete document and references can be found at www.ccs.ca or www.cachnet.org.…”

Ebstein’s Anomaly with Refractory Right-Side Heart Failure and Leg Ulcers

“…The efficacy of inhibitors of angiotensin-converting enzyme I (ACE I) in patients with Ebstein's anomaly and right-sided heart failure remains controversial. Medical management of arrhythmias should be individualized and combined with operative or catheter-based interventions [1], as emphasized by the Canadian Cardiovascular Society (CCS) Consensus Conference guidelines [2] on heart failure.…”

“…The CCS recommends surgical intervention if of the following indications are present [2]: A limited exercise capacity (NYHA grade III-IV); an increase in heart size (a cardiothoracic ratio greater than 65%); significant cyanosis (a resting oxygen saturation of less than 90%); severe symptomatic tricuspid valve regurgitation; and a transient ischemic attack or a stroke. The principal surgical procedures are placement of a tricuspid valve prosthesis, tricuspid valve replacement, heart transplantation, and heart-lung transplantation.…”

“…The prognosis of patients with Ebstein's malformation varies. McElhinney et al [2] found that, of neonates with Ebstein's anomaly, 20-40% did not survive for 1 month, and fewer than 50% survived to 5 years [3]. Celermajer et al [4] reviewed 220 cases of Ebstein's anomaly, with 1-34 years of follow-up.…”

Eisenmenger syndrome: current perspectives

“…26 Prophylactic or routine venesection to maintain a hematocrit level within an arbitrary predetermined level (hematocrit <65%) is not indicated. 27,28 This is one of the major misconceptions in the management of patients with cyanotic CHD and routine venesection can be harmful to these patients, as they can result in iron deficiency, reduced exercise tolerance, impaired oxygen transport capacity due to "relative anemia", and may increase the risk of cerebrovascular events. 29,30 Venesection should only be offered to patients with moderate-to-severe hyperviscosity symptoms (Table 3) due to significant secondary erythrocytosis (Hb level well above 24 g/dL) in the absence of volume depletion/dehydration.…”