Congenital Heart Disease in the Adult: A Review with Internet-Accessible Transesophageal Echocardiographic Images

The number of adults recognized with congenital heart disease (CHD) has increased dramatically over the past five decades because of significant advances in diagnosis and medical and surgical care. At the moment, the population of adults with CHD (ACHD) in the United States is estimated at approximately one million (1). For the first time, the number of adults with congenital cardiovascular malformations equals the number of children with these disorders. With additional refinements in surgical techniques and definitive repair at an earlier age, this patient group is likely to increase even further. Survival rates in CHD are influenced by many factors, including year of birth, age at diagnosis, complexity of the pathology, and whether the lesion(s) has been palliated or surgically corrected (Table 1) (1). As survival and life expectancy continue to improve, a growing number of unoperated, palliated, and “repaired” individuals require surgical interventions or other procedures related or unrelated to their heart disease. The care of these patients is becoming more frequent in all surgical settings, including tertiary care facilities, ambulatory centers, and labor and delivery suites.Table 1: Survival Rate from Year of Birth (1940–2000) by Complexity of Congenital Heart DiseaseAdults with CHD may come to the attention of anesthesiologists for various indications including: Cardiac surgery for the first time (for either palliation or definitive surgery) Cardiac reoperation for further palliation or definitive correction after palliative surgery Cardiac surgery for management of residua, complications of prior intervention, or conversion of a priori repair to a modern, potentially more favorable, strategy Noncardiac surgery or other nonsurgical procedures in the presence of uncorrected, palliated, or corrected lesions. Anesthesia and surgery may carry an increased risk for adverse events during emergent or elective procedures in these patients. This is particularly the case in those with cyanosis, pulmonary hypertension, rhythm disturbances, and significant hemodynamic abnormalities. Recommendations from organizations such as the American College of Cardiology (1) and the Canadian Cardiovascular Society (2–4) suggest that these patients should be cared for by cardiac anesthesiologists who have specialized training or extensive experience in the field. However, anesthesia care providers with such advanced expertise may not always be available. The challenge in caring for these patients is further magnified by the fact that there is a heterogeneous population. Individuals may present at any time with a bewildering array of structural variations, each with specific physiologic perturbations and hemodynamic consequences, and situations that require sophisticated perioperative care. The spectrum of CHD ranges widely from relatively mild defects seen in isolation to lesions of moderate to severe complexity typically characterized by several coexistent malformations. An important objective in caring for ACHD is to diminish cardiac-related morbidity and avoid adverse perioperative events. Of utmost importance in this mission is having a basic understanding of the native anatomy, physiology, surgical strategies, and late outcome of the defect under consideration. The primary goal of this article is to present a general overview of the most common congenital cardiovascular defects as applied to the adult age group, with a focus on anatomy, physiology, and long-term outcome (Table 2). To facilitate this review, representative images of the various congenital pathologies, as displayed by transesophageal echocardiography (TEE), accompany this contribution. The graphics are accessible as digital clips on the Web site of Anesthesia & Analgesia (www.anesthesia-analgesia.org), and we hope the clips will serve as reference material for those involved in the care of these patients. The images are labeled according to the American Society of Echocardiography/Society of Cardiovascular Anesthesiologists guidelines (5). We have made a significant effort to display most of the echocardiographic images as obtained in the population of focus, the adult patient. This imaging modality has provided significant contributions to the care of patients with structural congenital cardiovascular pathology, and we emphasize the benefits of this technology. The TEE imaging planes and information of interest for each of the lesions considered are listed in Table 3 as a guide to those who may want to become more familiar with the applications of this imaging approach to CHD. Epicardial echocardiography contributed significantly in the early experience of intraoperative imaging in patients with CHD; however, it is used primarily in patients when TEE is not feasible.Table 2: Congenital Heart Disease: Long term OutcomeTable 2: ContinuedTable 2: ContinuedTable 3: Transesophageal Echocardiography (TEE) in the Evaluation of Congenital Heart DiseaseTable 3: ContinuedFor an in-depth review of ACHD and the applications of TEE in these patients, the reader is referred to several comprehensive resources on the subject (6–11). The anesthestic considerations and management issues of CHD are beyond the scope of this article and have been addressed elsewhere (12–15). We have divided this manuscript into a discussion of simple and complex lesions, with “complex” defined as the presence of more than one congenital malformation often requiring surgical intervention. Simple Lesions Atrial Septal Defects (ASD) Anatomy and Physiology. Defects in the interatrial septum or ASDs comprise 7%–10% of all congenital cardiac anomalies (16). These defects account for nearly a third of all structural defects detected in adults, occurring more commonly in female patients than in males (17). Although classification of ASDs is primarily based on their location, characterization of interatrial communications is important in view of the incidence of associated anomalies and their impact on surgical management. Several types of defects are recognized including the following: 1) Ostium secundum or fossa ovalis defect (75% of ASDs) is the result of a deficiency in the septum in the region of the fossa ovalis (near or at the mid-aspect of the interatrial septum). Varying degrees of mitral valve prolapse and/or mitral regurgitation can occur in the adult related to myxomatous degeneration (18–21). 2) Ostium primum defect (15% of ASDs), regarded as a form of atrioventricular septal (canal) defect, involves a deficiency in the inferior aspect of the interatrial septum. Abnormalities of the atrioventricular valves occur most commonly in the form of a commissure or “cleft” in the anterior mitral leaflet potentially accompanied by variable degrees of valvular regurgitation. 3) Sinus venosus defect (10% of ASDs) is usually located in the superior aspect of the atrial septum, inferior to the junction of the superior vena cava and right atrium. This defect, also known as superior vena cava-type of sinus venosus ASD, is more common than its counterpart the inferior vena cava-type of defect (located posteriorly at the inferior vena to right atrial junction). These interatrial communications are frequently associated with anomalous pulmonary venous drainage (80%–90% of cases) from the right lung (22). 4) Coronary sinus defects (relatively rare) consist of a communication between the left atrium and mouth of coronary sinus. These defects are commonly associated with unroofing of the coronary sinus and a persistent left superior vena cava (LSVC) that drains directly into the left atrium (23,24). Other entities that may not be routinely considered in the classification of ASDs but may allow for interatrial shunting include a patent foramen ovale (PFO) at one end of the spectrum (25) and a confluent or common atrium at the other. Patency of the foramen ovale has been reported in as many of 25% of patients (26). In recent years, the presence of a PFO has been associated with the pathogenesis of migraine headaches (27,28). The potential for right-to-left shunting allowed by an incompetent flap of the fossa ovalis may be a risk factor in some patients for paradoxical embolization and cerebrovascular morbidity. A common atrium is characterized by complete or near-complete absence of the interatrial septum and is seen most frequently within the context of complex CHD. A direct communication between the atrial chambers allows for pulmonary venous blood to enter the right atrium. The magnitude of interatrial shunting relates to the size of the defect, relative ventricular compliances, and pulmonary artery pressures. A clinically significant defect results in right-sided volume overload characterized by right atrial, right ventricular, and pulmonary artery dilation. The abnormally increased pulmonary blood flow may be a long-term risk factor for the development of pulmonary vascular changes in a small number of patients (5%–10%). Several factors are considered in evaluating the need for intervention. These include the magnitude of the shunt or pulmonary flow (Qp) to systemic flow (Qs) ratio (also known as Qp:Qs) and concerns regarding the potential detrimental effects of chronic right ventricular volume overload. Further factors that influence the management approach include the presence or potential for atrial arrhythmias, risks for the development of pulmonary hypertension, pulmonary vascular obstructive disease, paradoxical embolization, and right ventricular failure. It is important to recognize that physiologic changes in left ventricular compliance and aging may account for unfavorable increases in the degree of left-to-right shunting, exacerbation of symptomatology, and development of right heart failure in the adult. Long-Term Outcome. Primary suture or patch closure of ASDs during childhood provides excellent operative results and nearly normal long-term survival (29,30). Surgical mortality is rare for isolated secundum defects in the current medical era. However, an increased risk is recognized in older patients and those with more than mild increases in pulmonary vascular resistance. As a rule, younger patients have a better outlook after repair (29–31). However, recent data have demonstrated that ASD closure is beneficial even in patients older than 50 or 60 yr (32). Both retrospective studies and prospective clinical trials suggested improved 10-yr survival in patients older than the age of 40 yr treated surgically (95%) compared with those treated medically (84%) (30,33,34). Atrial arrhythmias may be seen especially after the third decade of life. Late repair, after age 41 yr, does not appear to reduce the incidence of rhythm disorders (29). A management strategy that combined defect closure with arrhythmia surgery (Cox/Maze procedure) has been reported to be of benefit in these patients (35). Closure of these defects by the transcatheter route is becoming a widespread alternative to the surgical approach (36–39). Outcomes appear to be good, with successful closure that is generally safe (40). Minimally invasive surgical techniques using a lateral thoracotomy or limited sternotomy have been developed for patients who are not candidates for interventional device closure. This surgical approach has become an attractive option for patients, with better postoperative recovery and improved cosmetic results (41,42). The development of robotic techniques has helped reduce both incision size and overall postoperative trauma. Closure of ASDs has been performed via an endoscopic approach safely and effectively (43). In this study, quality of life outcome measures were superior in patients who received endoscopic surgery as compared with traditional sternotomy and mini-thoracotomy; however, further outcome studies are needed to evaluate the safety and efficacy of this approach. TEE. The identification and comprehensive characterization of ASDs by transthoracic echocardiography in the adult may be limited in some instances by poor acoustic windows. Transesophageal evaluation should be considered a complementary imaging modality in ascertaining or confirming the presence, size, and location of the defect in these patients. The mid-esophageal (ME) four-chamber and bicaval views are particularly useful in the examination of the atrial septum by two-dimensional imaging and color Doppler (Fig. 1 and Table 3) (see video clips 1–3 at www.anesthesia-analgesia.org). Additional benefits of this technology include assessment of the severity of associated atrioventricular valve regurgitation, chamber enlargement and ventricular function (transesophageal and transgastric views). Concomitant defects such as anomalous pulmonary venous drainage can also be defined by a combination of imaging planes. TEE has been shown to be of benefit during transcatheter closure by assisting in the selection of appropriate devices and monitoring during placement (Fig. 2 and Table 3) (see video clips 4 and 5 at www.anesthesia-analgesia.org) (44). Intraoperative benefits during cardiac procedures include documentation of the adequacy of the repair, exclusion of potential problems related to the intervention, and facilitation of cardiac de-airing. Obstruction to systemic or pulmonary venous flow, as well as erroneous diversion of systemic venous drainage to the left atrium, can be recognized by TEE.Figure 1.: Atrial Septal Defects. Top: Secundum atrial septal defect. Mid-esophageal four-chamber view demonstrating the large interatrial communication, with superior and inferior rims of atrial septal tissue bordering the centrally located defect. Color Doppler interrogation shows predominantly left-to-right shunting. RA = right atrium; RV = right ventricle. Middle: Primum atrial septal defect. Left: Mid-esophageal four-chamber view showing the defect in the inferior aspect of the interatrial septum. Arrow indicates the location of the atrial septal defect. LA = left atrium; LV = left ventricle. Right: Color flow Doppler interrogation demonstrates atrial level left-to-right shunting through the atrial septal defect. Bottom: Sinus venosus atrial septal defect. Mid-esophageal bicaval view showing a large atrial communication at the superior aspect of the interatrial septum, underneath the entrance of the superior vena cava into the right atrium. A dilated right pulmonary artery is shown in its short axis as it courses in perpendicular fashion behind the superior vena cava. RPA = right pulmonary artery; SVC = superior vena cava.Figure 2.: Atrial Septal Defects. Device Closure. Left: Transcatheter closure of atrial septal defect. Foreshortened mid-esophageal four-chamber view obtained during transcatheter closure of a secundum atrial septal defect. The clamshell device (arrow) is noted to be in good position in the interatrial septum. The legs of the device straddle both aspects of the interatrial septum. RA = right atrium. Right: Dislodged clamshell occluder device. Mid-esophageal four-chamber view with probe anteflexion shows an echogenic foreign body in the left ventricle. Embolization of the atrial septal defect occluder device (arrow) resulted in this being dislodged at the tips of the mitral valve leaflets. The patient required emergency surgery for device retrieval and closure of the interatrial communication. LA = left atrium; LV = left ventricle; RV = right ventricle.Color flow mapping contributes to the evaluation of interatrial shunting and atrioventricular valve competency. Contrast echocardiography with agitated saline can enhance the identification of small atrial level shunts, as microbubbles are readily apparent in the left atrium even when a very small number move across the defect (45,46). Ventricular Septal Defects (VSD) Anatomy and Physiology. VSDs are the most common of all congenital cardiac anomalies, excluding a studies suggest that these defects account for nearly of all at the ventricular level can be in isolation or may be seen in the context of other structural malformations. with VSDs are frequently than are those with defects usually require surgical attention during childhood for related to heart failure or pulmonary Although VSDs have a more frequent of closure in children small and defects may also even in classification have been for VSDs The classification noted of types is based on the location of the defect. However, in some the rims of the defect may beyond the of a region of the ventricular septum to 1) the most common of are located in the septum, inferior to the level of the these defects are associated with valve or septal valve tissue that may flow through or the defect. 2) defects of are located within the of the ventricular septum, including the anterior and and defects can the of a septum, surgical closure 3) or (also known as defects of are in the region that to the of the and valve is generally These defects may have associated or to regurgitation This results from of valvular by the septum. 4) defects of occur in the of the ventricular septum in to the atrioventricular atrioventricular valve anomalies frequently Defects that may be in with VSDs include a and right ventricular in the form of valve or anomalous right ventricular An communication may also be present in complex of CHD and in types of These communications allow for shunting at the ventricular The physiologic of this are by the size of the defect, of shunting, and relative of the pulmonary and systemic vascular VSDs are also in physiologic as either ventricular than left ventricular or defects or ventricular the defect is the flow across it is usually This is often the case with small the defect is large and the magnitude of the shunt is on the ratio between the pulmonary and systemic vascular A pulmonary vascular in the context of a to a large left-to-right The pulmonary blood flow in results in increased left ventricular In to the classification of VSDs according to their location or characterization of this malformation in of size and likely hemodynamic is useful as pulmonary to systemic ratio and The defect to hemodynamic right ventricular pulmonary vascular and left ventricular size are typically pulmonary to systemic ratio more than and of to These lesions may be associated with volume overload and degree of pulmonary is typically as are left atrial and left ventricular dilation. These defects are common than defects in the adult. ratio more than and more than In most patients a defect of this magnitude to the development of pulmonary vascular obstructive disease Long-Term Outcome. Surgical closure of VSDs early in childhood results in excellent with survival into generally Surgical in older children may be associated with left ventricular function and increased left ventricular regarded as may not be This has to regarding the need for surgical intervention. In a long-term of adults with small closure in during adult however, complications in 25% of this These complications regurgitation and rhythm with atrial being most common A number of individuals with moderate defects may relatively adult life when related to ventricular dilation. Heart more than and an increased pulmonary artery are clinical of an adverse (17). of patients with VSDs characterized by pulmonary vascular obstructive disease and in the of the ventricular level shunt These patients can into but typically have an overall survival The of the clinical of is known as made in Several the term to include pulmonary at systemic related to increased pulmonary vascular with or shunting through a large This the associated with pulmonary vascular changes and related to a in the of an or level in these patients relates to problems associated with chronic and such as cerebrovascular and the Other complications include and The long-term for patients with this is better than in those with other of pulmonary vascular pathology, such as primary pulmonary However, life expectancy is significantly with a reported survival of at yr, at yr, and at yr associated with poor include increased right ventricular and significant of patients from ventricular with have combined heart and lung and lung has as an Surgical closure of VSDs is the magnitude of the increase in pulmonary vascular is not However, the ratio of the pulmonary to systemic vascular the risk associated with surgical is In a of adult patients with postoperative problems were the pulmonary vascular postoperative pulmonary the is with right ventricular failure occurring commonly In patients with defects associated with regurgitation, late results after surgical closure of the defect and are generally A survival of at yr has been reported in patients, with from failure and from reoperation to be and at yr Transcatheter closure has been in for both postoperative and VSDs with excellent closure rates and TEE. The of TEE in the evaluation of patients with VSDs has been well (Table 3) Transesophageal examination allows for of the location and size of the defect and of chamber and in the of associated anomalies, and provides for identification of ventricular septal in to the assessment of the valve for and/or regurgitation that allow for a comprehensive examination of the ventricular septum include the four-chamber view that from the anterior to the and the transgastric short axis view 3 and 4 and Table 3) (see video clips at www.anesthesia-analgesia.org). Doppler color flow imaging allows for of the and magnitude of the ventricular shunt and identification and of associated regurgitation. and Doppler can be used to the flow across the and to an of and pulmonary artery In the presence of the across the is with a relatively across the ventricular In the absence of pulmonary the across the as by Doppler can be used to according to the as Ventricular Septal Defects. Top: ventricular septal defect. Left: Mid-esophageal four-chamber view demonstrating a deficiency in the septum with a ventricular septal defect. LA = left atrium; LV = left ventricle; RA = right atrium; RV = right ventricle. Right: Color Doppler interrogation across the defect left-to-right shunting. Bottom: ventricular septal defect. Left: Mid-esophageal axis view showing a ventricular septal defect. The of this defect to the valves is Right: Color Doppler demonstrates ventricular level Ventricular Septal Defects. Left: ventricular septal defect. Mid-esophageal four-chamber view showing left-to-right shunting through a small ventricular septal defect at the inferior aspect of the patch LV = left ventricle; RV = right ventricle. Middle: ventricular septal defect. Mid-esophageal four-chamber view demonstrating a large ventricular septal defect the level of the atrioventricular Right: Mid-esophageal four-chamber view with color flow Doppler showing left to right ventricular shunting.

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