P e rc u t a n e o u s Op t i o n s f o r H e a r t Failure in Adults with Congenital Heart Disease Darren Mylotte, MB MRCPI, MDa, Giuseppe Martucci, MD, FRCPCa,*, Nicolo Piazza, MD, PhD, FRCPC, FESCa, Doff McElhinney, MDb KEYWORDS Adult congenital heart disease Percutaneous intervention Transcatheter intervention Heart failure
KEY POINTS
INTRODUCTION Heart failure (HF) is a complex biochemical and physiologic response to the inability of the heart to meet the metabolic demands of the body. Hemodynamically, HF results from a decrease in cardiac output, a function of both heart rate and stroke volume (SV). The SV is determined by preload, afterload, and ventricular contractility, and derangement of 1 or a combination of these elements can reduce the SV. In patients with congenital heart disease (CHD), a variety of cardiac and extracardiac anatomic defects can initiate pathophysiologic change, culminating in reduced SV and HF. These defects can be categorized as either ventricular pressure- or volume-loaded states. Transcatheter interventions play a critical role in the treatment of HF in adult patients with CHD (ACHD). These procedures complement the more traditional medical and surgical therapies to form
a comprehensive therapeutic approach for the treatment of HF in ACHD. An in-depth review of all transcatheter interventions performed by the adult interventional congenital cardiologist is beyond the scope of this article. Hence, the most commonly encountered CHD lesions associated with HF and their transcatheter treatments are described: atrial septal defect (ASD), ventricular septal defect (VSD), and patent ductus arteriosus (PDA); right-sided obstructive and regurgitant lesions, including pulmonary regurgitation (PR) and stenosis; and left-sided obstructive lesions, such as aortic coarctation. These lesions are organized anatomically into those that predominantly affect the right ventricle (RV) and those affecting the left ventricle (LV). Of course, this practical and simplified approach does not account for the principles of ventricular interdependence, nor does it take into consideration that many patients with CHD have complex anatomies, with more than 1 lesion competing for physiologic dominance.
a Department of Interventional Cardiology, McGill University Health Centre, Royal Victoria Hospital, 687, Pine Avenue West, Montre´al H3A-1A1, Que´bec, Canada; b Department of Pediatrics, Langone Medical Center, New York University, 550 First Avenue, New York, NY 10016, USA * Corresponding author. McGill Adult Unit for Congenital Heart Disease (MAUDE Unit), 687 Pine Avenue West, Montre´al, Que´bec H3A 1A1, Canada. E-mail address: [email protected]
Heart Failure Clin 10 (2014) 179–196 http://dx.doi.org/10.1016/j.hfc.2013.09.018 1551-7136/14/$ – see front matter Ó 2014 Elsevier Inc. All rights reserved.
heartfailure.theclinics.com
Transcatheter interventions have evolved considerably in recent years and now play a key role in the treatment of heart failure (HF) in adult patients with congenital heart disease (CHD). These innovative procedures, when performed successfully and in a timely fashion, can lead to reversal of ventricular dilatation and dysfunction. Percutaneous interventions complement medical and surgical therapies and together form a comprehensive therapeutic approach for the treatment of HF in adults with CHD.
180
Mylotte et al LESIONS PREDOMINANTLY AFFECTING THE RV ASDs ASDs are the most common form of CHD in adults, accounting for up to one-third of all cases. They may be classified anatomically (Table 1): ostium primum in the lower part of the atrial septum; ostium secundum in the region of the fossa ovalis; sinus venosus with overriding of the atrial septum by the superior or inferior vena cava; and coronary sinus defects. Ostium secundum are the most common defects (75%), followed by ostium primum (15%), and sinus venosus defects (10%). ASDs may present as isolated lesions or may be associated with other cardiac abnormalities.1 ASDs allow shunting of blood from 1 atrium to the other. The direction and magnitude of the shunt are determined by the size of the ASD and the relative compliance of the ventricles.2 Small shunts (1.5:1 with LV systolic or diastolic dysfunction; (3) Qp/Qs greater than 1.5:1 if pulmonary artery pressure is less than two-thirds systemic pressure and PVR is less than two-thirds SVR; and (3) history of infective endocarditis.5 VSDs inducing progressive aortic valve disease are also considered for closure. Furthermore, adults with previous surgical VSD closure require close surveillance, because residual defects occur in up to 30% of cases.68 In such patients, LV volume overload and progressive aortic regurgitation are indications for reintervention. VSD closure is contraindicated in patients with severe irreversible pulmonary arteriolar hypertension.5 Transcatheter VSD closure Although surgical VSD closure is considered to be the gold standard treatment of VSD, it can be associated with significant morbidity and mortality.69,70 Transcatheter VSD closure devices In 1987, Lock and colleagues71 reported experience using the Rashkind double-umbrella device in patients who were declined surgical VSD closure. More recently, the Amplatzer muscular
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and perimembranous VSD occluders (St Jude Medical, St Paul, MN) have been introduced.72–74 These more operator-friendly devices are specifically designed for VSD closure, are available in a range of sizes, and can be recaptured and repositioned.75 Safety, efficacy, and complications of transcatheter VSD closure Several case series have reported acceptable safety and efficacy data for the Amplatzer muscular and perimembranous VSD occluders.75–81 Procedural mortality is rare. Successful delivery of the device can be achieved in more than 95% of cases.75,77,78,80 Both devices show impressive efficacy, with immediate complete VSD closure rates of 60% or more, increasing to 90% to 100% at 6 months follow-up.72,73,77–82 Residual shunts are usually trivial/mild with curative surgery required in 0.7%.75 Significant complications associated with transcatheter VSD closure occur in up to 6.5% of cases.75 Vascular complications were reported in 0.3% in a large European registry.75 The reported incidence of persistent complete heart block with transcatheter perimembranous VSD closure is between 1.07% and 3.9%, and up to 2.4% require implantation of a permanent pacemaker.75,77,78,80 Late heart block, not infrequently occurring up to 3 years after transcatheter VSD closure, has recently prompted a redesign of the Amplatzer perimembranous VSD occluder.83 Device embolization has been reported in up to 1.2% of cases.75 Up to 3.4% and 6.5% of patients develop aortic or tricuspid regurgitation, respectively.75 In most cases, valvular regurgitation is trivial/mild, with infrequent surgery required for aortic insufficiency.75,81 Outcomes after successful percutaneous VSD closure show significant improvement in NYHA functional class. In addition, there is significant remodeling of the volume-loaded LV, such that, in most patients, the LV dimension and LV function return to normal.80,81
PDA In fetal life, the ductus arteriosus is a vital conduit, connecting the left pulmonary artery with the descending aorta (distal to the left subclavian artery). The ductus permits pulmonary arterial blood to bypass the unexpanded lungs, enter the descending aorta, and perfuse the lower body. Normally, the ductus arteriosus closes within 48 hours of birth, but in some cases, spontaneous closure does not occur, and there is continuous left to right shunting from the aorta to the pulmonary artery. PDA accounts for approximately 10% of all CHD
lesions and occurs more commonly in pregnancies complicated by perinatal hypoxemia or maternal rubella. The most common associated lesions are VSDs or ASDs. A PDA rarely closes spontaneously after infancy.84 Again, the hemodynamic impact of a PDA depends on the magnitude of shunting. Small PDAs result in minimal left to right shunting, are unlikely to causes symptoms, and hence have a negligible impact on life expectancy. Moderatesize PDAs may remain undetected during childhood and present with congestive HF or arrhythmia in adulthood.85 Large shunts tend to present early with LV dilatation and failure; and if uncorrected, pulmonary arterial hypertension ensues, with reversal of shunt direction when the PVR exceeds the SVR.86 The diagnosis of PDA may be suggested by clinical features and confirmed by transthoracic echocardiography. The anatomic features of the PDA, the magnitude of the shunt, and the PVR can be evaluated at cardiac catheterization. Indications for PDA closure PDA closure is indicated in the presence of LV enlargement and in the presence of pulmonary arterial hypertension if there is net left to right shunting (Box 1).5 Although some clinicians believe that transcatheter PDA closure is deemed to be a reasonable strategy in asymptomatic patients with small PDA because of the increased risk of infective endarteritis, this approach is controversial and most do not advocate prophylactic closure of small silent PDA. Previous endarteritis is however, an indication for PDA closure. PDA closure is not
Box 1 PDA: indications for closure and reported transcatheter complications Indications for closure Closure is indicated if 1. There is evidence of left atrial or LV enlargement 2. The presence significant pulmonary arterial hypertension 3. Net left to right shunting 4. History of infective endocarditis 5. Considered reasonable in asymptomatic patients with small PDA because of the risk of infective endocarditis Complications of transcatheter closure Embolization: infrequent (
KEY POINTS
INTRODUCTION Heart failure (HF) is a complex biochemical and physiologic response to the inability of the heart to meet the metabolic demands of the body. Hemodynamically, HF results from a decrease in cardiac output, a function of both heart rate and stroke volume (SV). The SV is determined by preload, afterload, and ventricular contractility, and derangement of 1 or a combination of these elements can reduce the SV. In patients with congenital heart disease (CHD), a variety of cardiac and extracardiac anatomic defects can initiate pathophysiologic change, culminating in reduced SV and HF. These defects can be categorized as either ventricular pressure- or volume-loaded states. Transcatheter interventions play a critical role in the treatment of HF in adult patients with CHD (ACHD). These procedures complement the more traditional medical and surgical therapies to form
a comprehensive therapeutic approach for the treatment of HF in ACHD. An in-depth review of all transcatheter interventions performed by the adult interventional congenital cardiologist is beyond the scope of this article. Hence, the most commonly encountered CHD lesions associated with HF and their transcatheter treatments are described: atrial septal defect (ASD), ventricular septal defect (VSD), and patent ductus arteriosus (PDA); right-sided obstructive and regurgitant lesions, including pulmonary regurgitation (PR) and stenosis; and left-sided obstructive lesions, such as aortic coarctation. These lesions are organized anatomically into those that predominantly affect the right ventricle (RV) and those affecting the left ventricle (LV). Of course, this practical and simplified approach does not account for the principles of ventricular interdependence, nor does it take into consideration that many patients with CHD have complex anatomies, with more than 1 lesion competing for physiologic dominance.
a Department of Interventional Cardiology, McGill University Health Centre, Royal Victoria Hospital, 687, Pine Avenue West, Montre´al H3A-1A1, Que´bec, Canada; b Department of Pediatrics, Langone Medical Center, New York University, 550 First Avenue, New York, NY 10016, USA * Corresponding author. McGill Adult Unit for Congenital Heart Disease (MAUDE Unit), 687 Pine Avenue West, Montre´al, Que´bec H3A 1A1, Canada. E-mail address: [email protected]
Heart Failure Clin 10 (2014) 179–196 http://dx.doi.org/10.1016/j.hfc.2013.09.018 1551-7136/14/$ – see front matter Ó 2014 Elsevier Inc. All rights reserved.
heartfailure.theclinics.com
Transcatheter interventions have evolved considerably in recent years and now play a key role in the treatment of heart failure (HF) in adult patients with congenital heart disease (CHD). These innovative procedures, when performed successfully and in a timely fashion, can lead to reversal of ventricular dilatation and dysfunction. Percutaneous interventions complement medical and surgical therapies and together form a comprehensive therapeutic approach for the treatment of HF in adults with CHD.
180
Mylotte et al LESIONS PREDOMINANTLY AFFECTING THE RV ASDs ASDs are the most common form of CHD in adults, accounting for up to one-third of all cases. They may be classified anatomically (Table 1): ostium primum in the lower part of the atrial septum; ostium secundum in the region of the fossa ovalis; sinus venosus with overriding of the atrial septum by the superior or inferior vena cava; and coronary sinus defects. Ostium secundum are the most common defects (75%), followed by ostium primum (15%), and sinus venosus defects (10%). ASDs may present as isolated lesions or may be associated with other cardiac abnormalities.1 ASDs allow shunting of blood from 1 atrium to the other. The direction and magnitude of the shunt are determined by the size of the ASD and the relative compliance of the ventricles.2 Small shunts (1.5:1 with LV systolic or diastolic dysfunction; (3) Qp/Qs greater than 1.5:1 if pulmonary artery pressure is less than two-thirds systemic pressure and PVR is less than two-thirds SVR; and (3) history of infective endocarditis.5 VSDs inducing progressive aortic valve disease are also considered for closure. Furthermore, adults with previous surgical VSD closure require close surveillance, because residual defects occur in up to 30% of cases.68 In such patients, LV volume overload and progressive aortic regurgitation are indications for reintervention. VSD closure is contraindicated in patients with severe irreversible pulmonary arteriolar hypertension.5 Transcatheter VSD closure Although surgical VSD closure is considered to be the gold standard treatment of VSD, it can be associated with significant morbidity and mortality.69,70 Transcatheter VSD closure devices In 1987, Lock and colleagues71 reported experience using the Rashkind double-umbrella device in patients who were declined surgical VSD closure. More recently, the Amplatzer muscular
187
Mylotte et al
188
and perimembranous VSD occluders (St Jude Medical, St Paul, MN) have been introduced.72–74 These more operator-friendly devices are specifically designed for VSD closure, are available in a range of sizes, and can be recaptured and repositioned.75 Safety, efficacy, and complications of transcatheter VSD closure Several case series have reported acceptable safety and efficacy data for the Amplatzer muscular and perimembranous VSD occluders.75–81 Procedural mortality is rare. Successful delivery of the device can be achieved in more than 95% of cases.75,77,78,80 Both devices show impressive efficacy, with immediate complete VSD closure rates of 60% or more, increasing to 90% to 100% at 6 months follow-up.72,73,77–82 Residual shunts are usually trivial/mild with curative surgery required in 0.7%.75 Significant complications associated with transcatheter VSD closure occur in up to 6.5% of cases.75 Vascular complications were reported in 0.3% in a large European registry.75 The reported incidence of persistent complete heart block with transcatheter perimembranous VSD closure is between 1.07% and 3.9%, and up to 2.4% require implantation of a permanent pacemaker.75,77,78,80 Late heart block, not infrequently occurring up to 3 years after transcatheter VSD closure, has recently prompted a redesign of the Amplatzer perimembranous VSD occluder.83 Device embolization has been reported in up to 1.2% of cases.75 Up to 3.4% and 6.5% of patients develop aortic or tricuspid regurgitation, respectively.75 In most cases, valvular regurgitation is trivial/mild, with infrequent surgery required for aortic insufficiency.75,81 Outcomes after successful percutaneous VSD closure show significant improvement in NYHA functional class. In addition, there is significant remodeling of the volume-loaded LV, such that, in most patients, the LV dimension and LV function return to normal.80,81
PDA In fetal life, the ductus arteriosus is a vital conduit, connecting the left pulmonary artery with the descending aorta (distal to the left subclavian artery). The ductus permits pulmonary arterial blood to bypass the unexpanded lungs, enter the descending aorta, and perfuse the lower body. Normally, the ductus arteriosus closes within 48 hours of birth, but in some cases, spontaneous closure does not occur, and there is continuous left to right shunting from the aorta to the pulmonary artery. PDA accounts for approximately 10% of all CHD
lesions and occurs more commonly in pregnancies complicated by perinatal hypoxemia or maternal rubella. The most common associated lesions are VSDs or ASDs. A PDA rarely closes spontaneously after infancy.84 Again, the hemodynamic impact of a PDA depends on the magnitude of shunting. Small PDAs result in minimal left to right shunting, are unlikely to causes symptoms, and hence have a negligible impact on life expectancy. Moderatesize PDAs may remain undetected during childhood and present with congestive HF or arrhythmia in adulthood.85 Large shunts tend to present early with LV dilatation and failure; and if uncorrected, pulmonary arterial hypertension ensues, with reversal of shunt direction when the PVR exceeds the SVR.86 The diagnosis of PDA may be suggested by clinical features and confirmed by transthoracic echocardiography. The anatomic features of the PDA, the magnitude of the shunt, and the PVR can be evaluated at cardiac catheterization. Indications for PDA closure PDA closure is indicated in the presence of LV enlargement and in the presence of pulmonary arterial hypertension if there is net left to right shunting (Box 1).5 Although some clinicians believe that transcatheter PDA closure is deemed to be a reasonable strategy in asymptomatic patients with small PDA because of the increased risk of infective endarteritis, this approach is controversial and most do not advocate prophylactic closure of small silent PDA. Previous endarteritis is however, an indication for PDA closure. PDA closure is not
Box 1 PDA: indications for closure and reported transcatheter complications Indications for closure Closure is indicated if 1. There is evidence of left atrial or LV enlargement 2. The presence significant pulmonary arterial hypertension 3. Net left to right shunting 4. History of infective endocarditis 5. Considered reasonable in asymptomatic patients with small PDA because of the risk of infective endocarditis Complications of transcatheter closure Embolization: infrequent (
