Adult congenital heart disease





Atrial septal defects



Anatomy and imaging of the atrial septum


The atrial septum is close to the TEE transducer position and is well visualized, as the normal orientation is perpendicular to the ultrasound beam. From a single high TEE probe position, the entire atrial septum can be examined by starting in a four-chamber view and then incrementally rotating the image plane to the bicaval view at approximately 90 to 120 degrees rotation. 3D imaging is especially helpful for visualization of the entire atrial septum and positioning of transcatheter closure devices.



Fig 7.1


Diagram of atrial septal components showing foramen ovale (FO), septum primum (Sept 1°), left atrium (LA), left ventricle (LV), fossa ovalis, superior limbic bands (SLB) and inferior limbic bands (ILB), atrioventricular septum (AVS), right atrium (RA), right ventricle (RV). (Adapted from Keane JF, Lock JE, Fyler DC (eds.): Nadas Pediatric Cardiology, 2e, Philadelphia, 2006, Elsevier/Saunders.)



Fig 7.2


Diagram of atrial septum showing several types of atrial septal defects. (Adapted from Keane JF, Lock JE, Fyler DC (eds.): Nadas Pediatric Cardiology, 2e, Philadelphia, 2006, Elsevier/Saunders.)



Fig 7.3


From high TEE position, fossa ovalis (FO) is seen in both four-chamber (A) and bicaval (B) views. At rotation angle between 60 and 90 degrees, opening between septum primum and septum secundum (arrow) or patent foramen ovale is seen. LA = left atrium, RA = right atrium, SP = septum primum, SS = septum secundum, FO = fossa ovalis.



Fig 7.4


In midesophageal position, orthogonal views at 110 degrees (left) and 20 degrees (right) will be used to construct 3D image. Pyramidal truncated data set (3D Zoom, QLAB; Philips, Medical Systems) includes entire atrial septum (see Fig 7.5 ).



Fig 7.5


Using multiplanar reconstruction modality base (QLAB; Philips, Medical Systems), initial 3D image (left frame) shows atrial septum from left atrial perspective. Rotating image along its horizontal axis (middle frame) allows imaging of atrial septum from right atrial perspective; proximity of ascending aorta can be appreciated, crucial concept in periprocedural septal puncture. Subsequent rotation of image along its vertical axis allows better appreciation of atrial septum from left atrial perspective. In all three frames, black arrow indicates fossa ovalis.



Fig 7.6


In this midesophageal view at 114 degrees, there is persistence of fetal remnants; Eustachian Valve and Chiari network. Red arrow indicates flow into right atrium from IVC. As can be seen, color Doppler signal (red arrow) is directed by these structures to fossa ovalis. In utero, this allows oxygenated blood returning from placenta to cross through patent foramen ovale to left atrium.



Fig 7.7


In left panel, midesophageal view at approximately 110 degrees shows PFO (arrow). Right panel is corresponding 3D image. In the video, bubbles are seen passing from the RA to LA indicative of a shunt through the PFO.



Fig 7.8


At left, color Doppler shows flow through PFO. Arrow indicates flow convergence on left atrial side; both color and spectral Doppler (right) indicate that flow is from left atrium to right atrium.



Fig 7.9


3D color Doppler shows hemispherical shape of flow acceleration (arrow). On right, multiplanar reconstruction is used to calculate dimension of PFO.



Fig 7.10


In patients in whom PFO is suspected but saline contrast study is negative, there are several diagnostic strategies. In panels A and B , probe is in midesophageal position with slight turning to patient’s left; color Doppler evidence of left-to-right shunt is confirmed by presence of “negative contrast” (black arrow) in right atrium. In panels C through F, first saline contrast study is unequivocally negative; however, injection of contrast with Valsalva maneuver is followed quickly by release of Valsalva, subsequent increase in right atrial pressure, and appearance of microbubbles in left atrium; red arrow in video indicates negative contrast from IVC flow.



Fig 7.11


In another patient with elevated right atrial pressure secondary to pulmonary hypertension, color Doppler is indicative of right atrial to left atrial flow.





Patent foramen ovale


Intraoperative transesophageal echocardiography (TEE) in a patient undergoing coronary artery bypass grafting demonstrated a patent foramen ovale (PFO).



Fig 7.12


View of left atrium (LA) and right atrium (RA) from high esophageal position at 69 degrees rotation demonstrates PFO at limbus of fossa ovalis with color Doppler showing small jet of flow from left to right (arrow).



Fig 7.13


Injection of agitated saline in peripheral vein opacifies right heart due to effect of microbubbles. Microbubbles in hand-agitated saline do not pass through pulmonary capillaries, leaving left heart unopacified in absence of intracardiac shunt. In this case, magnified image at about 70 degrees rotation shows passage of contrast from right to left across patent foramen ovale (arrow).



Fig 7.14


Intraoperative view, with right atrium (RA) opened, demonstrates PFO in interatrial septum.




Comments


A PFO can be demonstrated on TEE in 20% to 25% of adults. The PFO typically is best seen from a high esophageal view of the atrial septum, with the transducer rotated between 60 and 90 degrees. The PFO is seen at the junction of the secundum septum (covering the fossa ovalis) and the primum septum. In most patients the PFO is small and functions as a “flap valve,” with no flow across the septum when atrial pressures are low and left atrial pressure is slightly higher than right atrial pressure. With a transient elevation in right atrial pressure, for example with Valsalva maneuver, the flap valve opens and blood can flow from right to left. With chronic elevation in atrial pressure, the PFO may become stretched with a defect between the right and left atrium allowing flow, even at rest. A small PFO is typically a benign incidental finding without associated clinical symptoms or signs. However, there is an association between the presence of a PFO and cryptogenic stroke, with studies in progress testing the hypothesis that closing the PFO will decrease the risk of recurrent stroke.


Suggested reading




  • 1.

    Asrress KN, Marciniak M, Marciniak A, Rajani R, Clapp B: Patent foramen ovale: the current state of play, Heart 101(23): 1916–1925, 2015.


  • 2.

    Gupta SK, Shetkar SS, Ramakrishnan S, Kothari SS: Saline contrast echocardiography in the era of multimodality imaging—importance of “bubbling it right”. Echocardiography 32(11): 1707–1719, 2015.




Atrial septal aneurysm


This 48-year-old woman with recurrent neurologic events was found to have an atrial septal aneurysm with evidence of right-to-left shunting on intracardiac echocardiography with agitated saline used for right heart contrast.



Fig 7.15


In high TEE view at about 90 degree of rotation bulging of atrial septum into RA is seen. Curvature of atrial septum exceeds 1.5 cm and persists in both systole and diastole, consistent with diagnosis of atrial septal aneurysm (ASA). See also small patent foramen ovale.



Fig 7.16


Color Doppler demonstrates left-to-right flow across PFO.



Fig 7.17


Right atrial angiography shows opacified right atrium with septum bulging into left atrium. With elevation of right atrial pressure during angiogram, septal curvature reverses (arrows) (see Fig 7.15 ).



Fig 7.18


Intraoperative view from opened right atrium shows central septum (arrow) bulging into right atrium.



Fig 7.19


At surgery, with right atrium open, large atrial septal aneurysm and small atrial septal defect were plicated and closed using pericardial patch.




Comments


In addition to a PFO, this patient has an atrial septal aneurysm, defined as transient bulging of the fossa ovalis region greater than 1.5 cm in the absence of chronically elevated left or right atrial pressures. The presence of an atrial septal aneurysm is associated with a higher risk of embolic stroke than a PFO alone, most likely related to the high (>90%) prevalence of fenestrations of the septum in patients with a septal aneurysm. In patients with recurrent neurologic events despite adequate medical therapy, PFO closure may be considered, either surgically or using a percutaneously inserted closure device.


Suggested reading




  • 1.

    McGrath ER, Paikin JS, Motlagh B, et al: Transesophageal echocardiography in patients with cryptogenic ischemic stroke: a systematic review, Am Heart J 168(5):706–712, 2014.




Secundum atrial septal defect


This 50-year-old man presented with increasing shortness of breath on exertion. After a negative pulmonary evaluation, he underwent echocardiography that demonstrated a secundum atrial septal defect (ASD).



Fig 7.20


PA chest radiograph shows cardiomegaly with prominent right atrial border and enlarged right pulmonary artery (arrow).



Fig 7.21


On lateral chest radiograph, right ventricular enlargement is evident with opacification of retrosternal space by enlarged right ventricle.



Fig 7.22


Chest CT demonstrates severe right ventricular and right atrial enlargement.



Fig 7.23


TEE four-chamber view shows enlarged right ventricle and atrium, although atrial septum appears intact in this image plane.



Fig 7.24


Slight anteflexion of probe, with image plane now including aortic valve, demonstrates ASD with left-to-right flow and defect diameter of 18 mm. Arrow indicates pulmonary artery catheter in right atrium.



Fig 7.25


Pulsed Doppler confirms left-to-right flow in both systole and diastole.



Fig 7.26


After sternotomy, right atrial appendage was noted to be redder than usual, consistent with shunting of oxygenated left atrial blood. Through open right atrium, 2 × 1 cm ASD was identified (left frame). In addition, smaller but separate defect was noted adjacent to tendon of Todaro (at origin of coronary sinus) (center). Both defects were closed with single pericardial patch, with postoperative image demonstrating patch across defect (right frame). Patient had atrial flutter 5 days postoperatively and was easily cardioverted back into sinus rhythm. However, he subsequently had recurrent atrial flutter that was treated with radiofrequency catheter ablation of caval tricuspid isthmus.




Comments


The most common type of atrial septal defect (ASD) is a secundum ASD, with the defect located centrally in the septum typically measuring ≥1 cm diameter. Although most ASDs are diagnosed and treated in childhood, a substantial number are not recognized until young adulthood, with a few diagnosed only later in life, as in this case.


The TEE features of an atrial septal defect are the consequence of right-sided volume overload. Blood flows left to right across the atrial defect so that the right heart pumps a larger stroke volume than the left heart. The severity of shunting is measured as the pulmonary flow (Qp) to systemic flow (Qs) ratio, where 1 is normal. A Qp:Qs ratio >1.5:1 is associated with progressive right atrial and right ventricular enlargement. In addition, ventricular septal curvature is reversed with “paradoxical” septal motion. Pulmonary hypertension is unusual with a secundum ASD. TTE may demonstrate the atrial defect itself with color Doppler showing left-to-right flow. A contrast study can be performed when right heart enlargement is present and images of the atrial septum are suboptimal.


TEE provides better images of the interatrial septum and is helpful when percutaneous closure is planned, to measure the size of the defect and evaluate the rim of tissue that will anchor the device.


Suggested reading




  • 1.

    Brickner ME, Hillis LD, Lange RA: Congenital heart disease in adults. First of two parts, N Engl J Med 342:256–263, 2000.


  • 2.

    Brickner ME, Hillis LD, Lange RA: Congenital heart disease in adults. Second of two parts, N Engl J Med 342:334–342, 2000.


  • 3.

    Faletra FF, Pedrazzini G, Pasotti E, et al: 3D TEE during catheter-based interventions, JACC Cardiovasc Imaging 7(3):292–308, 2014.




Primum atrial septal defect


This 34-year-old asymptomatic woman was incidentally noted to have a murmur, which prompted echocardiography. This study revealed a large primum ASD with left-to-right flow and a Qp:Qs of 2.5:1. In addition, a cleft anterior leaflet of the mitral valve with moderate mitral regurgitation was demonstrated.



Fig 7.27


Chest radiography shows enlargement of right and left atrium, right ventricle, and pulmonary artery, with increased pulmonary blood flow.



Fig 7.28


In four-chamber view in diastole on 2D (left) and color (right) crest of ventricle septum is seen (arrow) with open tricuspid and mitral valve on either side. With mitral and tricuspid valves open and large atrial defect, all four cardiac chambers have equal pressures in diastole.



Fig 7.29


In same view in systole, tricuspid and mitral valve are closed. Arrowhead indicates ASD. Moderate-to-severe mitral regurgitation is seen, in part due to cleft anterior mitral valve leaflet.



Fig 7.30


Short-axis view of mitral valve obtained with probe retroflexed just distal to gastroesophageal junction, demonstrates cleft (asterisk) in anterior mitral leaflet (AML). On right, regurgitant flow is seen through cleft (arrow).



Fig 7.31


At surgery, opening right atrium reveals absence of primum septum, and two atrioventricular valves.



Fig 7.32


Cleft in anterior mitral leaflet is demonstrated (asterisk).



Fig 7.33


At surgery, ostium primum defect was closed with pericardial patch.



Fig 7.34


Anterior mitral leaflet (AML) was repaired by suturing cleft leaflet and placing 30-mm annuloplasty ring.



Fig 7.35


Postprocedure four-chamber view shows ASD patch with no residual flow.



Fig 7.36


Postprocedure short-axis view of mitral valve in diastole (A) , and in the same orientation as Fig 7.30 , shows repair of cleft leaflet (white arrow) . In systole (B) , the repair of cleft leaflet (white arrow) is again seen, with some residual central mitral regurgitation seen on color Doppler ( C , red arrow ).




Comments


A primum ASD is seen in the base of the septum, adjacent to the atrioventricular valve plane and, in effect, is a partial atrioventricular canal defect. The defect is best seen in a four-chamber view, both on 2D imaging and with color Doppler. These defects are large, typically requiring surgical closure with placement of a patch. Many patients have associated abnormalities of the atrioventricular valve, most commonly a cleft anterior mitral valve leaflet. Some cleft leaflets can be repaired by approximation of the two segments, but others require replacement if the valve is deformed or if there is excessive tension when the segments are sutured together. The echocardiographer should also carefully evaluate for a ventricular septal defect (VSD) in patients with a primum ASD.


Suggested reading




  • 1.

    Mahmood F: Perioperative transesophageal echocardiography: Current status and future direction, Heart 102:1159–1167, 2016.


  • 2.

    Randolph GR, Hagler DJ, Connolly HM, et al: Intraoperative transesophageal echocardiography during surgery for congenital heart defects, J Thorac Cardiovasc Surg 124:1176–1182, 2002.




Primum atrial septal defect with previous atrioventricular canal defect repair


The patient, a 29-year-old woman, who had an atrioventricular canal defect repaired as a child, presents with increasing shortness of breath, and on TTE was found to have a residual left-to-right shunt at the atrial level, most likely from a residual atrial septal defect. Mitral regurgitation and a persistent left superior vena cava were also present.



Fig 7.37


Complex anatomy is demonstrated in this diastolic midesophageal four-chamber view. Red dotted line indicates tricuspid annulus, whereas white dotted line indicates mitral annulus. White arrow indicates interatrial septum, and asterisk, primum ASD. Large coronary sinus (CS) can be seen emptying into right atrium, just above tricuspid valve.



Fig 7.38


In this midesophageal long-axis view during systole, left and center panels show central jet of mitral regurgitation (arrow), and flow in left ventricular outflow tract. Right panel demonstrates so-called “goose-neck” deformity in which distance from left ventricular apex to posterior mitral annulus (white dotted line) is 20% to 25% shorter than distance from left ventricular apex to aortic annulus (red dotted line).



Fig 7.39


In this deep transgastric image, left frame is in diastole. In systolic frame on right, dashed line indicates that continuous wave Doppler can be aligned reasonably parallel to flow to measure gradient in left ventricular outflow tract (LVOT).



Fig 7.40


On left is 3D TEE of mitral valve from left atrial perspective, in which partial cleft in anterior leaflet of mitral valve is noted (arrow) . On right is valve as seen in surgical field.



Fig 7.41


In this midesophageal image rotated to patient’s left, left atrial appendage, left upper pulmonary vein (LUPV), and persistent left superior vena cava (red arrow) are seen. On right, agitated saline is seen in persistent left superior vena cava after injection of saline in left antecubital vein.



Fig 7.42


Intraoperatively, atrial septal defect was closed successfully with pericardial patch, cleft in anterior mitral leaflet repaired, and mitral ring annuloplasty placed. However, as seen in this midesophageal long-axis view, ring annuloplasty (white arrows) is displaced anteriorly, producing narrowing and turbulence in left ventricular outflow tract.



Fig 7.43


From deep transgastric view, late-peaking systolic left ventricular outflow tract velocity of 4.2 m/s, corresponding to peak gradient of 69 mm Hg, was measured.



Fig 7.44


Patient was returned to cardiopulmonary bypass. Because of outflow tract gradient, annuloplasty ring was removed. After second separation from cardiopulmonary bypass, there was no longer any evidence for obstruction in outflow tract with normal blue laminar flow seen in this transgastric apical view. Only mild to moderate amount of mitral regurgitation (MR) remained.




Comments


This case illustrates the utility of intraoperative TEE guidance during surgery for complex congenital heart disease. Recognition of subaortic obstruction due to the annuloplasty ring impinging on the narrow LV outflow tract in this patient with a history of an AV canal defect resulting in prompt correction at the same surgical procedure.


Suggested reading




  • 1.

    De Mey N, Couture P, Denault AY, et al: Subaortic stenosis after atrioventricular septal defect repair, Anesth Analg 113:236–238, 2011.


  • 2.

    Kutty S, Smallhorn JF: Evaluation of atrioventricular septal defects by three-dimensional echocardiography: benefits of navigating the third dimension, J Am Soc Echocardiogr 25(9): 932–944, 2012.




Sinus venosus atrial septal defect


This 34-year-old man presented with increasing fatigue, shortness of breath, and palpitations. After an episode of atrial fibrillation, he underwent echocardiography, which showed a probable ASD with moderate right ventricular and right atrial enlargement. TEE demonstrated the anatomy of the sinus venosus defect, with a maximum diameter of 2.4 cm. Right heart catheterization showed normal right heart pressures with a Qp:Qs ratio of 2.5:1.



Fig 7.45


From high TEE position at 0 degrees, communication ( arrow , ASD) between superior vena cava (SVC) and left atrium (LA) is seen (A) . Color flow demonstrates low-velocity flow through this region (B).



Fig 7.46


Color flow demonstrates flow between two atria at superior aspect of atrial septum.

Jan 2, 2020 | Posted by in CARDIOLOGY | Comments Off on Adult congenital heart disease

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