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Postoperative Imaging in Cyanotic Congenital Heart Diseases: Part 2, Complications

¹ Department of Radiology, Complejo Hospitalario Universitario Juan Canalejo, Xubias de Arriba 84, 15006 La Coruña, Spain.
² Department of Pediatric Cardiology, Complejo Hospitalario Universitario Juan Canalejo, La Coruña, Spain.

Received February 23, 2007; revised June 19, 2007;

 
Address correspondence to E. Rodríguez (esther.rodriguez{at}mundo-r.com).

CME

This article is available for CME credit. See www.arrs.org for more information.

Abstract

Top Abstract Introduction Palliative Surgeries Corrective Surgeries Conclusion References

 
OBJECTIVE. The purpose of this article is to illustrate the MRI appearance of postoperative complications in the surgical procedures most commonly performed to correct cyanotic congenital heart disease.

CONCLUSION. The radiologist must be familiar with the morphologic and functional MRI appearances of surgical complications in patients with palliated or repaired cyanotic congenital heart disease to deliver an accurate diagnosis on which to base management decisions.

Keywords: cardiac surgery • complications • congenital heart disease • cyanosis • hemodynamics • MRI • shunts

Introduction

Top Abstract Introduction Palliative Surgeries Corrective Surgeries Conclusion References

 
Most patients with cyanotic congenital heart diseases who live to adulthood have undergone surgical palliation or repair. New technical developments in MRI, along with the complexity of the surgery itself and the possible appearance of subsequent complications, have led to the increasingly common use of MRI in the postoperative evaluation of these patients [1].

The postoperative complications that appear in connection with cyanotic congenital heart disease constitute a new diagnostic challenge for the radiologist, requiring familiarity with the anatomic and functional complexities of palliative and corrective surgical procedures.

This article shows MR images of a wide range of postoperative complications after palliative procedures (systemic arterial and venous–to–pulmonary artery shunts, pulmonary artery banding, and Rastelli procedure) in patients with cyanotic congenital heart disease and the surgery performed to correct tetralogy of Fallot and dextroposed transposition of the great arteries (D-TGA).

Palliative Surgeries

Top Abstract Introduction Palliative Surgeries Corrective Surgeries Conclusion References

 
Systemic–to–Pulmonary Artery Shunts
The purpose of surgically shunting the systemic and pulmonary arteries is to increase pulmonary blood flow in the interim before permanent reconstruction.

Systemic–to–pulmonary artery shunts are inserted in patients with cyanotic congenital heart disease involving pulmonary stenosis or atresia and as the first stage in procedures to treat different forms of single ventricle. At present, the modified Blalock-Taussig shunt is the type most commonly used. The complications associated with this procedure include shunt obstruction (Fig. 1A) or stenosis with secondary lung perfusion deficiency (Fig. 1B), stenosis, and pseudoaneurysm (Fig. 1A) at the pulmonary or subclavian artery anastomosis; pulmonary hypertension; and left ventricular dysfunction secondary to volume overload [2].

View larger version (57K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1A —6-month-old male infant with pulmonary atresia and increasing cyanosis after placement of modified Blalock-Taussig shunt. Reconstructed shaded surface display gadolinium-enhanced 3D MR angiogram reveals left modified Blalock-Taussig shunt occlusion (arrow) and false aneurysm (arrowhead) at proximal anastomosis with left subclavian artery. Ascending aorta is also observed to be dilated.

View larger version (140K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 1B —6-month-old male infant with pulmonary atresia and increasing cyanosis after placement of modified Blalock-Taussig shunt. Coronal thin-slab (10-mm) maximum-intensity-projection image shows almost no perfusion in left lung (star) and slight perfusion deficit in right upper lobe (arrowhead).

One of the most significant advantages of the modified Glenn shunt is that, unlike systemic arterial–to–pulmonary shunts, it does not increase the volume load on the ventricle. The chief limitation to this technique, however, is that an anatomically adequate pulmonary vascular bed with low resistance is mandatory for it to be successful. The goal of diagnostic evaluation before proceeding to the full Fontan procedure (total cavopulmonary anastomosis) is to identify hypoplasia of the pulmonary arteries and stenosis of the venous anastomosis (Fig. 2A, 2B, 2C, 2D). Complications of Fontan circulation include thromboembolic events related to atrial enlargement (Fig. 3) and arrhythmias, atrioventricular valve regurgitation (Fig. 4A), stenosis of the cavopulmonary anastomosis or pulmonary arteries (Figs. 4B and 4C), pulmonary arteriovenous malformations, collateral veins or arteries, ventricular dysfunction, ischemic complications due to surgical coronary artery lesions, and protein-losing enteropathy [3, 4].

View larger version (131K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2A —23-year-old man with modified Glenn shunt for tricuspid atresia presenting with increasing cyanosis. MRI was prescribed to evaluate shunt status. Coronal cine MR image of heart shows low-signal-intensity jet flow (arrowhead) across anastomosis of superior vena cava and right pulmonary artery.

View larger version (157K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2B —23-year-old man with modified Glenn shunt for tricuspid atresia presenting with increasing cyanosis. MRI was prescribed to evaluate shunt status. Reconstructed maximum-intensity-projection (MIP) (B) and shaded surface display (C) gadolinium-enhanced 3D MR angiograms show modified Glenn shunt with stenosis at right pulmonary artery anastomosis (arrows), dilatation of superior vena cava (stars), and flow through azygos and hemiazygos venous system (arrowheads, C) to divert blood away from stenosis.

View larger version (31K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2C —23-year-old man with modified Glenn shunt for tricuspid atresia presenting with increasing cyanosis. MRI was prescribed to evaluate shunt status. Reconstructed maximum-intensity-projection (MIP) (B) and shaded surface display (C) gadolinium-enhanced 3D MR angiograms show modified Glenn shunt with stenosis at right pulmonary artery anastomosis (arrows), dilatation of superior vena cava (stars), and flow through azygos and hemiazygos venous system (arrowheads, C) to divert blood away from stenosis.

View larger version (126K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 2D —23-year-old man with modified Glenn shunt for tricuspid atresia presenting with increasing cyanosis. MRI was prescribed to evaluate shunt status. Coronal MIP image shows low perfusion in left lung and perfusion defect in right upper lobe (arrow). Blood can also be observed to flow through azygos and hemiazygos veins (arrowheads).

View larger version (131K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 3 —12-year-old boy presenting with dyspnea on exertion whose single functional ventricle was palliated in infancy via original Fontan procedure. Sonographic findings (not shown) suggested atrial thrombus. Reconstructed coronal maximum-intensity-projection image shows large right atrium (star) and patent conduit (arrow) between right atrium and main pulmonary artery (arrowhead).

View larger version (127K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4A —13-year-old girl presenting with dyspnea on exertion who had undergone extracardiac Fontan operation for tricuspid atresia. Axial steady-state free precession cine MR image of heart shows extracardiac Fontan circulation (arrow) to bypass hypoplastic right ventricle; ventricular septal defect; and small signal void (arrowhead) extending from mitral valve into left atrium in systole, corresponding to mitral valve regurgitation.

View larger version (140K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4B —13-year-old girl presenting with dyspnea on exertion who had undergone extracardiac Fontan operation for tricuspid atresia. Reformatted contrast-enhanced 3D maximum-intensity-projection (MIP) image shows patent extracardiac conduit (arrow) between inferior vena cava and right pulmonary artery. Superior vena cava is connected to top of right pulmonary artery (Glenn anastomosis). After total cavopulmonary anastomosis, superior vena cava flow is directed mostly to right pulmonary artery, and inferior vena cava flow, to left pulmonary artery. Due to flow dynamics after this procedure and timing of imaging acquisition, there is paucity of contrast material in inferior vena cava and extracardiac conduit resulting in low perfusion in left lung (star).

View larger version (154K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 4C —13-year-old girl presenting with dyspnea on exertion who had undergone extracardiac Fontan operation for tricuspid atresia. Reformatted contrast-enhanced 3D MIP image obtained after B reveals left pulmonary artery stenosis (arrowhead).

Banding complications include erosion of the band in the pulmonary arterial lumen with secondary stenosis or pseudoaneurysm (Fig. 5A, 5B), distal migration of the band with branch pulmonary artery stenosis, pulmonary insufficiency secondary to dilatation of the pulmonary annulus, and subaortic obstruction due to the reduction of ventricular volume and induction of progressive ventricular hypertrophy [5] (Fig. 6A, 6B).

View larger version (137K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5A —8-year-old boy with neonatal pulmonary artery banding to correct large ventricular septal defect and right aortic arch. Second operation was performed because band erosion was suspected. Status after closure of ventricular septal defect and pulmonary artery debanding was assessed on MRI. Reformatted maximum-intensity-projection (A) and shaded surface display (B) gadolinium-enhanced 3D MR angiograms reveal false aneurysm at main pulmonary artery (arrows) due to band erosion.

View larger version (59K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 5B —8-year-old boy with neonatal pulmonary artery banding to correct large ventricular septal defect and right aortic arch. Second operation was performed because band erosion was suspected. Status after closure of ventricular septal defect and pulmonary artery debanding was assessed on MRI. Reformatted maximum-intensity-projection (A) and shaded surface display (B) gadolinium-enhanced 3D MR angiograms reveal false aneurysm at main pulmonary artery (arrows) due to band erosion.

View larger version (166K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6A —15-year-old girl presenting with increasing cyanosis whose double-outlet right ventricle and unrestricted pulmonary flow were palliated in infancy via pulmonary artery banding. Coronal maximum-intensity-projection image shows banding-induced stenosis at main pulmonary artery (arrow).

View larger version (132K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 6B —15-year-old girl presenting with increasing cyanosis whose double-outlet right ventricle and unrestricted pulmonary flow were palliated in infancy via pulmonary artery banding. Short-axis cine MR image at mid ventricle reveals right ventricular myocardial hypertrophy (arrowheads) and leftward displacement of interventricular septum due to pressure overload induced by pulmonary artery banding.

Indeed, stenosis of the extracardiac conduit (with or without regurgitation) (Fig. 7A, 7B, 7C, 7D) is inevitable and left ventricular dysfunction is common. Other possible developments include right ventricular dysfunction secondary to abnormal pressure, volume load related to conduit dysfunction, obstruction of the baffle from the left ventricle to the aortic valve, and branch pulmonary artery stenosis [6].

View larger version (104K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7A —15-year-old boy presenting with mild cyanosis on exertion; patient had undergone Rastelli operation for dextroposed transposition of the great arteries. Anterior 3D shaded surface display MR angiogram shows narrowed and tortuous conduit (arrows) from right ventricle (star) to pulmonary artery (arrowhead).

View larger version (137K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7B —15-year-old boy presenting with mild cyanosis on exertion; patient had undergone Rastelli operation for dextroposed transposition of the great arteries. Axial cine MR image shows low-signal-intensity jet flow (arrow) across conduit from right ventricle to pulmonary artery and artifactual signal loss from metallic sternal sutures (star).

View larger version (107K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7C —15-year-old boy presenting with mild cyanosis on exertion; patient had undergone Rastelli operation for dextroposed transposition of the great arteries. Phase velocity-encoded cine MR images on plane perpendicular to conduit during systole (C) and diastole (D). Phase-contrast images, in which flow is black during systole (arrow, C) and white during diastole (arrowhead, D), show reverse blood flow during cardiac cycle due to conduit regurgitation. Regurgitation rate calculated for this patient was 35%, and pressure gradient was found to peak at 40 mm Hg. Artifact (stars) produced by metallic sternal sutures also can be seen.

View larger version (112K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 7D —15-year-old boy presenting with mild cyanosis on exertion; patient had undergone Rastelli operation for dextroposed transposition of the great arteries. Phase velocity-encoded cine MR images on plane perpendicular to conduit during systole (C) and diastole (D). Phase-contrast images, in which flow is black during systole (arrow, C) and white during diastole (arrowhead, D), show reverse blood flow during cardiac cycle due to conduit regurgitation. Regurgitation rate calculated for this patient was 35%, and pressure gradient was found to peak at 40 mm Hg. Artifact (stars) produced by metallic sternal sutures also can be seen.

Top Abstract Introduction Palliative Surgeries Corrective Surgeries Conclusion References

Surgical repair via closure of the ventricular septal defect and relief of RVOT stenosis is the present treatment of choice in early infancy. The most common complication of surgical repair of RVOT obstruction is pulmonary valve regurgitation (Fig. 8A), which may lead to right ventricular dilatation, tricuspid regurgitation (Fig. 8B), arrhythmias, or biventricular dysfunction [7, 8]. Although the optimal timing for pulmonary valve replacement is still a matter of debate [9], the use of phase-contrast and cine MRI to accurately quantify pulmonary regurgitation and the right ventricular ejection fraction can often help identify when surgery can best be performed to prevent irreversible damage to the ventricle [8].

View larger version (148K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8A —20-year-old man with surgically repaired tetralogy of Fallot who presented with dyspnea on exertion. Sagittal steady-state free precession cine MR image shows jet flows in right ventricle (arrow) due to pulmonary valve regurgitation and in main pulmonary artery (arrowhead) due to residual stenosis. According to time–flow curves obtained from velocity-encoded cine MR images (not shown), gradient peak was 24 mm Hg and regurgitation fraction, 40%.

View larger version (141K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 8B —20-year-old man with surgically repaired tetralogy of Fallot who presented with dyspnea on exertion. Axial steady-state free precession cine MR image obtained during mild systole shows dilatation of right atrium and right ventricle and jet flow through tricuspid valve (arrowhead), indicative of tricuspid regurgitation.

View larger version (136K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9A —22-year-old woman presenting with dyspnea; patient had undergone surgical correction of tetralogy of Fallot. Imaging was performed to assess state of cardiopathy. Short-axis steady-state free precession cine MR image of right ventricular outflow tract (RVOT) during end-diastole shows aneurysmal dilatation of RVOT patch (arrow).

View larger version (125K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9B —22-year-old woman presenting with dyspnea; patient had undergone surgical correction of tetralogy of Fallot. Imaging was performed to assess state of cardiopathy. Short-axis inversion-recovery turbo field-echo MR image reveals delayed enhancement of RVOT (arrow) extending into right ventricular trabeculae (arrowhead).

View larger version (47K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 9C —22-year-old woman presenting with dyspnea; patient had undergone surgical correction of tetralogy of Fallot. Imaging was performed to assess state of cardiopathy. Reconstructed shaded surface display gadolinium-enhanced 3D MR angiogram shows left pulmonary artery to be cranially elongated, kinked at root (arrow), and distally dilatated (star), secondary to aneurysmal dilatation of RVOT. Roots of two branches of pulmonary artery are at obtuse angle to main artery.

View larger version (79K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 10 —24-year-old man presenting with mild hypoplasia of main pulmonary artery; patient had undergone surgical repair for tetralogy of Fallot. Imaging was performed to assess present status of repair. Posterior coronal 3D shaded surface display MR angiogram of roots of left and right pulmonary arteries shows residual segmental narrowing (arrow) of left pulmonary artery with distal dilatation and hypoplasia of main pulmonary artery.

View larger version (100K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 11A —20-year-old man presenting with increasing cyanosis; patient had undergone surgical repair for tetralogy of Fallot consisting of placement of synthetic conduit to connect main pulmonary artery to right ventricle. Reconstructed sagittal maximum-intensity-projection (MIP) (A) and coronal shaded surface display (B) 3D MR angiograms reveal short stenosis in distal conduit anastomosis (arrows) and aneurysmal dilatation of main pulmonary artery (arrowheads). According to time–flow curves obtained from velocity-encoded cine MR images (not shown), gradient peaked at 65 mm Hg.

View larger version (93K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 11B —20-year-old man presenting with increasing cyanosis; patient had undergone surgical repair for tetralogy of Fallot consisting of placement of synthetic conduit to connect main pulmonary artery to right ventricle. Reconstructed sagittal maximum-intensity-projection (MIP) (A) and coronal shaded surface display (B) 3D MR angiograms reveal short stenosis in distal conduit anastomosis (arrows) and aneurysmal dilatation of main pulmonary artery (arrowheads). According to time–flow curves obtained from velocity-encoded cine MR images (not shown), gradient peaked at 65 mm Hg.

Aortic valve or aortic root replacement is occasionally required to correct progressive aortic root dilatation (Fig. 12) with aortic regurgitation related to prior long-lasting aortic volume overloads and possibly to intrinsic properties of the arterial root [12].

View larger version (62K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 12 —24-year-old man with tetralogy of Fallot and atretic main pulmonary artery palliated in infancy via modified Blalock-Taussig shunt and repaired at age of 7 years with valved conduit homograft between right ventricle and pulmonary arteries. Reconstructed shaded surface display gadolinium-enhanced 3D MR angiogram shows patency of both right ventricle–to–pulmonary artery conduit (lower pair of arrowheads) and modified Blalock-Taussig shunt (top arrowhead), as well as aortic root aneurysm (arrow). Velocity-encoded cine MRI (not shown) revealed normal functioning of aortic valve and right ventricle–to–main pulmonary artery conduit.

Most D-TGA patients who survive to adulthood have undergone an atrial switch operation (Mustard or Senning procedure). Baffle leakage and baffle obstruction are complications that commonly appear after such procedures. Patients also frequently suffer right ventricular hypertrophy and enlargement (Fig. 13A, 13B) and right ventricular failure [1].

View larger version (116K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13A —17-year-old girl presenting with dyspnea on exertion; patient had undergone atrial switch operation (Mustard procedure) in infancy for dextroposed transposition of the great arteries. Axial cine MR image obtained during diastole shows isolation of mitral valve from pulmonary venous flow by atrial baffle and hypertrophy of systemic right ventricle outflow tract (arrowhead).

View larger version (103K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 13B —17-year-old girl presenting with dyspnea on exertion; patient had undergone atrial switch operation (Mustard procedure) in infancy for dextroposed transposition of the great arteries. Four-chamber cine MR view obtained during diastole shows enlargement (star) and hypertrophy (arrowheads) of systemic right ventricle with interventricular septal flattening (arrow) secondary to chronic volume overload. Volumetric MR quantification (not shown) gave right ventricular ejection fraction of 38%.

View larger version (73K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 14A —8-month-old male infant after arterial switch procedure (Jatene arterial switch procedure) with Lecompte maneuver to surgically repair dextroposed transposition of the great arteries. MRI was prescribed to evaluate surgical repair of cardiopathy. Axial cine MR images of pulmonary bifurcation obtained during systole (A) and diastole (B) reveal low-signal-intensity blood flow turbulence (arrow, B) in right pulmonary artery during diastole, secondary to stenosis. No significant difference was observed between peak flow values for right and left pulmonary arteries during cardiac cycle found by velocity-encoded cine MRI quantification (not shown).

View larger version (72K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 14B —8-month-old male infant after arterial switch procedure (Jatene arterial switch procedure) with Lecompte maneuver to surgically repair dextroposed transposition of the great arteries. MRI was prescribed to evaluate surgical repair of cardiopathy. Axial cine MR images of pulmonary bifurcation obtained during systole (A) and diastole (B) reveal low-signal-intensity blood flow turbulence (arrow, B) in right pulmonary artery during diastole, secondary to stenosis. No significant difference was observed between peak flow values for right and left pulmonary arteries during cardiac cycle found by velocity-encoded cine MRI quantification (not shown).

View larger version (62K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 14C —8-month-old male infant after arterial switch procedure (Jatene arterial switch procedure) with Lecompte maneuver to surgically repair dextroposed transposition of the great arteries. MRI was prescribed to evaluate surgical repair of cardiopathy. Oblique posterior reconstructed shaded surface display 3D MR angiogram reveals dilatation of aortic root (arrows).

View larger version (104K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 15A —6-month-old male infant with dextroposed transposition of the great arteries after arterial switch procedure (Jatene arterial switch procedure). Sonographic findings suggested left ventricular pseudoaneurysm. MRI was performed to obtain preoperative anatomic and functional information. Short-axis cine MR image obtained during diastole reveals bulging cavity (arrows) with narrow neck (arrowheads) connected to basal portion of left ventricle.

View larger version (113K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 15B —6-month-old male infant with dextroposed transposition of the great arteries after arterial switch procedure (Jatene arterial switch procedure). Sonographic findings suggested left ventricular pseudoaneurysm. MRI was performed to obtain preoperative anatomic and functional information. Reconstructed oblique maximum-intensity-projection (B) and shaded surface display (C) gadolinium-enhanced 3D MR angiograms show saccular outpouching arising from inferior wall of left ventricle (arrows).

View larger version (77K): [in this window] [in a new window] [as a PowerPoint slide]   Fig. 15C —6-month-old male infant with dextroposed transposition of the great arteries after arterial switch procedure (Jatene arterial switch procedure). Sonographic findings suggested left ventricular pseudoaneurysm. MRI was performed to obtain preoperative anatomic and functional information. Reconstructed oblique maximum-intensity-projection (B) and shaded surface display (C) gadolinium-enhanced 3D MR angiograms show saccular outpouching arising from inferior wall of left ventricle (arrows).

Top Abstract Introduction Palliative Surgeries Corrective Surgeries Conclusion References

References

Top Abstract Introduction Palliative Surgeries Corrective Surgeries Conclusion References

 

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This Article Abstract Figures Only Full Text (PDF) CME Credit Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via Google Scholar Google Scholar Articles by Soler, R. Articles by Raposo, I. Search for Related Content PubMed PubMed Citation Articles by Soler, R. Articles by Raposo, I. Social Bookmarking                      What's this? Hotlight (NEW!) What's Hotlight?