See Figure 49-3A.
See Figure 49-3B, right panel.
See Figure 49-3B, left panel.
See Figure 49-6C.
See Figure 49-6A.
See Figure 49-6D.
See Figure 49-6B.
It shows the sequence of steps used for the voltage mapping technique to predict the slow AV node location. The same equipment, type of catheters and steps were used as described in Figure 21.25 A-D. The total recording time was 20 minutes but the video was shortened to 7.5 minutes for optimal viewing. The first 3 min. 45 sec are shown at 4x speed at the end of which the reference was changed briefly from the 20-pole catheter to the His catheter to enable more voltage mapping beneath the His catheter area. Then 2x speed and normal speed are intermittently used at end of voltage data point collection. The propagation map at normal speed starts at 4 minutes and the wave front collision at site of predicted slow pathway during the propagation map is shown at 5 min 17 sec. The added hand written black circle predicts the cryo ablation target. At 5 min 50 seconds, the cryoablation applications are displayed followed by individual placement. Cryo-applications 1-6 (blue circles) were 1-1.5 min in duration and were unsuccessful (inducible AVNRT during application). These applications were placed anteriorly towards the tricuspid valve annulus and slightly outside the voltage mapping-predicted slow pathway bridge area because the atrial electrograms appeared more ideal (low voltage, high frequency). Therefore, the electrograms (using conventional anatomic / electrogram approach) were thought to be inconsistent with the voltage map. However, despite the less than optimal slightly greater amplitude electrograms, when cryo-application 7 was applied closer to the voltage mapping-predicted slow pathway, immediate block in the slow pathway was recorded (using the S1-S2 atrial stimulation technique) at less than one minute. This application and subsequent “bonus” ones 8-12 (orange) were placed during 4 minute applications. No inducible AVNRT was evident after cryo 7. (Courtesy of Christopher C. Erickson, MD Children's Hospital & Medical Center, Professor of Pediatrics, University of Nebraska Medical Center; and John Prusmack, St. Jude Medical S.C., Inc.who assisted in the data acquisition, refinement and display.)
NC/C ratio in systole is >2.8.
RV compromise is highly suspected.
As we sweep cephalad, the branching pulmonary artery is seen arising from the left ventricle, and the non-branching aorta is seen arising from the right ventricle, which lies anterior and rightward of the pulmonary artery. Of note, three vessels appear to branch from the pulmonary artery; these are the right and left pulmonary arteries and the ductus arteriosus.
The valgus is worse on the left than the right. Note the medial femoral condyles bumping into each other during the gait cycle.
This toddler with achondroplasia has genu varum with internal tibial torsion.
2D and color Doppler parasternal long-axis clip showing a posteriorly directed jet of mitral regurgitation extending >2 cm behind the mitral valve and a small jet of aortic regurgitation.
Cineangiogram through a pigtail catheter advanced retrograde to the descending thoracic aorta following PDA closure with a 6-6 Amplatzer Duct Occluder II demonstrates a well-seated device with no residual shunting. There is no evidence of aortic obstruction. Courtesy of Renelle George, MD (AngioAtlas.org)
Contrast is seen appearing in the left atrium after 3 cardiac cycles consistent with a positive contrast study.
The mital valve chordae and papillary muscles appear echobright secondary to ischemia.
There is a severely dilated left ventricle with severely depressed systolic function. The mitral valve chordae and papillary muscles appear echobright secondary to ischemia.
The left coronary artery (seen branching into the left anterior descending and circumflex coronary arteries) is seen arising from the pulmonary artery with retrograde flow into the pulmonary artery (blue color Doppler flow)
The left ventricle appears severley dilated with severely depressed systolic function. The mitral valve papillary muscles appear echobright secondary to ischemia.
His unsteadiness results in a significant increase in double-limb stance (usually 20% of the gait cycle) and a decrease in single-limb stance (usually 80%), compared to typical gait. (Video with permission of the Children's Orthopaedic Center, Los Angeles.)
Plain radiograph shows soft tissue swelling over the distal fibula and lytic changes at the metaphysis. MRI shows significant involvement of distal tibia and fibula and a large abscess infiltrating the surrounding soft tissues. Video of incision of drainage of the lesion.
Moderately depressed global ventricular function. Contrast was negative to rule out apical thrombosis.
Matching apical 4-chamber.
The tibia subluxes laterally on the femur during stance phase with each step. This is revealed as a momentary wobble (or thrust) at the knee during walking.
Multiple segments to the right and left lungs were supplied by aortopulmonary collaterals not demonstrated on this angiogram. Note the tubular hypoplasia centrally with mild discrete stenosis in both main branch pulmonary arteries.
A pigtail catheter is positioned in the aortic root retrograde from the femoral artery. With injection, the aortic valve is doming and while the aortic valve annulus is normal, there is a narrow jet of negative contrast washout through the effective orifice of the aortic valve. The ascending aorta is also dilated. There is no aortic insufficiency.
The left-sided AV valve appears more apically displaced consistent with a left-sided tricuspid valve. The septal leaflet appears tethered and shortened. The left atrium appears severely dilated. The (left-sided) right ventricle appears hypertrophied and trabeculated in appearance
There is a large vegetation noted on the septal leaflet of the tricuspid valve. Echo image courtesy of www.pedecho.org
The tricuspid valve appears atretic with a plate-like oriface.
Echo image courtesy of www.pedecho.org
The common AV valve appears largely committed to the left ventricle. The right ventricle appears moderately hypoplastic.
The common AV valve appears largely committed to the right ventricle. The left ventricle appears moderately hypoplastic.
Mild to moderate tricuspid regurgitation is noted secondary to elevated right ventricular pressures in the setting of pulmonary venous obstruction
There is severe hypertrophy of the ventricular septum with systolic anterior motion of the mitral valve causing severe dynamic left ventricular outflow tract obstruction
The leaflets demonstrate restricted excusion.
There is end systolic right atrial collapse and diastolic right ventricle collapse consistent with tamponade physiology.
The left ventricle and mitral valve are severely hypoplastic.
Note the swinging heart and the prominent right atrial wall collapse in late diastole.
Asterisks illustrate deep inter-recess spaces.
Stored fluoroscopy during deployment of a 20 mm Amplatzer Septal Occluder across the atrial septum. The left atrial disc is deployed first, followed by the center waist and right atrial disc.Courtesy of Renelle George, MD (AngioAtlas.org)
Stored fluoroscopy during release of a 20 mm Amplatzer Septal Occluder positioned across the atrial septum. The device is in good position following release. Courtesy of Renelle George, MD (AngioAtlas.org)
This child with a syrinx has asymmetric abdominal reflexes.
Stored fluoroscopy during balloon aortic valvuloplasty with a 7 mm x 3 cm Tyshak II balloon. The waist disappears completely at maximal inflation. A pacing catheter is positioned in the right ventricle to increase balloon stability during the inflation. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram via a pigtail catheter in the ascending aorta of a seven week-old male with a thickened, unicommissural aortic valve and severe aortic stenosis. There is no significant aortic valve regurgitation at baseline. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram through a pigtail catheter in the ascending aorta following balloon aortic valvuloplasty demonstrates trivial aortic valve regurgitation. Courtesy of Renelle George, MD (AngioAtlas.org)
Stored fluoroscopy during inflation of a 11 mm x 3 cm Tyshak II balloon across the pulmonary valve. There is near-complete resolution of the waist at maximal inflation. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram via a Berman catheter in the right ventricle of a 5-month-old female with moderate-to-severe valvar pulmonary stenosis demonstrates a hypertrophied right ventricle with qualitatively normal systolic function. The pulmonary valve is thickened and domes in systole. There is post-stenotic dilatation of the main pulmonary artery. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram via the Berman catheter in the main pulmonary artery following balloon pulmonary valvuloplasty demonstrates improved leaflet excursion. The dilated main pulmonary artery with confluent, good-sized branch pulmonary arteries are again visualized. There is no significant pulmonary regurgitation. Courtesy of Renelle George, MD (AngioAtlas.org)
There is a right aortic arch with an aberrant left subclavian artery arising from a bulbous Kommerell diverticulum. There is fixed mild tracheal narrowing at the level of the vascular ring. The aorta is labeled in red, the trachea in orange, and the pulmonary artery in blue.
There is a right dominant double aortic arch with mild to moderate extrinsizc compression of the mid to lower trachea at the level of the double arch.
The left pulmonary artery arises from the right pulmonary artery and extends leftward and posteriorly.
The anomalous muscle bundle is seen dividing the inflow and outflow RV chambers, similar to the angiographic findings.
There is late gadolinium enhancement in the same area on LGE imaging.
This young boy has a spinal cord tumor documented in Figure 5.49. During play, he has difficulty walking and does not move his neck in order to see things. Torticollis can be one of the earliest physical examination features for spinal cord tumors of the cervical spine.
2D echocardiographic apical four-chamber clip demonstrating thickened leaflets, restricted diastolic excursion, left atrial (LA) enlargement.
2D echocardiographic parasternal long-axis clip demonstrating thickened mitral valve leaflets with restricted diastolic motion and a ‘hockey-stick’ deformity of the anterior leaflet.
Child presented as nonweight bearing with history of left hip pain for 3 days. Radiographic analysis is negative. Ultrasound indicated the presence of a left hip effusion. Even under sedation, hip is flexed externally rotated and abducted.
(Courtesy of Joshua M. Abzug, MD.)
Note that this test is best performed while the patient is under anesthesia. (Courtesy of Joshua M. Abzug, MD.)
Cineangiogram via a pigtail catheter in the transverse arch of an adult male with recurrent coarctation of the aorta following end-to-end anastomosis in infancy demonstrates discrete coarctation just distal to the origin of the left subclavian artery. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram via a pigtail catheter in the transverse arch following balloon angioplasty with a 5 mm x 2 cm Tyshak mini balloon demonstrates significant improvement in the coarcted segment. There is no evidence of vascular injury. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram via a pigtail catheter in the transverse arch of a 7-week-old premature male demonstrates a severe, discrete coarctation of the aorta distal to the origin of the left subclavian artery. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram via pigtail catheter in the transverse arch following implantation of a 26 mm Max LD stent on an 18 mm BiB and post-dilation with an Atlas Gold balloon demonstrates a well-apposed stent with no evidence of vascular injury. Flow to the head and neck vessels is unobstructed. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram through a pigtail catheter advanced retrograde to the descending aorta following PDA closure with a 5mm x 4mm Nit-Occlud PDA coil demonstrates a well-seated device with no residual shunting. There is no evidence of aortic obstruction. Courtesy of Renelle George, MD (AngioAtlas.org)
The arch is hypoplastic, and arch flow is completely retrograde.
Some atrial septal tissue is seen bowing into the left atrium. There is mild right ventricular hypertrophy.
This young woman with hemiplegia has dynamic forefoot supination, secondary to spasticity of the anterior tibialis tendon. She is unable to dorsiflex her foot without flexing her hip and knee, demonstrating a positive confusion test.
Image courtesy of J. Chris Wilkinson, Texas Children's Hospital.
Our patient underwent bilateral talectomies and 6 weeks later had distal femoral extension osteotomies.
This seven-year-old girl with DD has crouched gait with severe knee and foot contractures.
It is important to note that Achilles tightness should be assessed with the foot inverted to lock the midfoot which otherwise may mask the true tightness, then first with the knee extended to keep the gastrocnemius tight, and then with the knee flexed to relax the gastrocnemius. In this child the tight Achilles prevents full ankle dorsiflexion, requiring motion through the midfoot to allow the foot to fully dorsiflex. The axis of movement of the midfoot is different from the ankle, and out-toeing is created.
Blocks can be used to level the pelvis. If blocks are not available, books or magazines can be stacked under the short limb to determine the leg length discrepancy.
Sitting allows the spine to be examined without the influence of leg lengths on the spinal alignment.
With the feet placed flat on the examination table and the knees flexed, the length of the tibia to the plantar aspect of the foot can also be compared between the two sides.
Notice the substantial anterior translation of the tibia on the femur in this patient with an incompetent ACL.
Inverting the foot prevents inadvertent motion in the midfoot. Flexing the knee relaxes the gastrocnemius muscle because it crosses both the knee and ankle joints.
Note the marked decrease in tissue Doppler early diastolic mitral annulus (e') velocities in patients with restrictive cardiomyopathy (typically below 8 cm/s), while patients with constrictive pericarditis have normal or increased e' velocities.
Cineangiogram through a pigtail catheter advanced retrograde to the ascending aorta in a patient with double outlet right ventricle with normally related great arteries and mitral valve hypoplasia status post extracardiac, non-fenestrated Fontan procedure demonstrates an unobstructed Damus-Kaye-Stansel anastomosis. There is no significant neoaortic or aortic valve regurgitation. There is a large coronary artery fistula arising from the left main coronary artery. Courtesy of Renelle George, MD (AngioAtlas.org)
This video represents a “sweep” through the anatomy of the infant with DOLV described in Figures 51.23 to 51.25 and Videos 51.1 and 51.2. The right ventricle and most of the ventricular septum have been “cropped” out of the image. Both great arteries are committed to the left ventricular chamber in this case, with only the most anterior aspect of the aortic root overriding the ventricular septal defect (not seen in the planes included in this video). A, anterior; F, “foot” or inferior; H, “head” or superior; L, left; P, posterior; R, right.
These simultaneous two-dimensional and color Doppler echocardiographic images were taken from a neonatal examination of a child with DOLV (also illustrated in Figs. 51.23 and 24). The outflow anatomy is typical of a conotruncal malformation and in this plane would be indistinguishable from a case of tetralogy of Fallot. There is mitral to aortic valve continuity (absence of subaortic conus) and the aortic annulus overrides the ventricular septum by roughly 50%. The color flow is especially prominent exiting the anterior right ventricle (RV) and entering the aorta, because the VSD provided the only outlet from the RV in this case.
These simultaneous two-dimensional and color Doppler echocardiographic images were taken from the same neonatal examination of a child with DOLV as in Video 51.1. Here the plane of sound has been angled anteriorly to demonstrate both great arteries (aorta to the patient's right). Both semilunar valves are committed to the left ventricular outlet, but neither has any significant subvalvar conus muscle. The color Doppler image (right panel) shows no obstruction to aortic flow. There was a mild gradient (mean = 20 mm Hg) through the pulmonary outlet. The red color signal in the main pulmonary artery (MPA) represents left to right flow entering the MPA from the ductal artery which remained patent at the time of the examination.
This is consistent with the diagnosis of slipped capital femoral epiphysis.
This child with hemiplegia has a dropped foot on the left side. Accommodation to clear the foot during swing phase is noted with increasing hip and knee flexion in swing phase.
This girl with spastic diplegia has a positive Duncan-Ely test. By flexing the knee rapidly, the hip will secondarily rise due to increasing contracture/spasticity in the rectus femoris tendon.
Left ventricular mid-short axis view.
Apical 4-chamber view.
This first branch is the left brachiocephalic artery. It divides into the left common carotid artery and the left subclavian artery. Note that the right common carotid artery can be seen coursing rightward just after the take-off of the brachiocephalic artery.
This first branch is the right brachiocephalic artery. The branch divides into the right common carotid artery and the right subclavian artery.
In systole, turbulent flow out the left ventricular outflow tract is begins at the level of the membrane. A narrow jet of aortic insufficiency is noted in diastole.
Left ventricular function appears normal and the aortic valve is normal in appearance.
The obstruction extends for approximately 1.5 cm from immediately below the aortic valve.
While the bicuspid nature of the valve cannot be identified from this view, the cusps are clearly seen to “dome”, resulting in a reduced effective valve orifice.
As is most common, the right and left coronary cusps are fused to create a large cusp with a thickened raphe at the site of the fused commissure. The valve opens with the “fish mouth” appearance characteristic of a bicuspid aortic valve.
The origin of the left coronary artery from the left coronary sinus is also demonstrated.
This hemiplegic boy has an equinovarus foot deformity marked by equinus positioning and fixed varus positioning in stance phase; likely due to spasticity/contracture of the posterior tibialis tendon.
This boy with cerebral palsy has an equinovarus deformity of his right foot. During gait, he has a foot drop and must have his leg abducted in order to clear it in swing phase. The tight Achilles leads to equinus positioning and back knee gait in stance phase.
Severe right pulmonary artery (RPA) stenosis in a patient who had repair of anomalous left coronary originating from the mid-RPA. Balloon occlusion is performed with the angiographic catheter in the proximal LPA to occlude flow distally forcing contrast into the tightly stenotic RPA.
Selective left pulmonary artery (LPA) injection in a patient with proximal LPA stenosis. With the frontal plane detector angled at 16 degrees cranial/6 RAO, the proximal LPA stenosis is completely foreshortened (see Video 16-4A). This assures the operator that the orthogonal view fully depicts the stenosis and that measurement of this segment will be accurate.
This boy with facioscapulohumeral dystrophy has difficulty with shoulder abduction. Winging of the scapula is noted.
Cineangiograms through a Berman angiocatheter in the Glenn circuit, distal conduit and inferior vena cava of a patient with double outlet right ventricle with normally related great arteries and mitral valve hypoplasia status post extracardiac, non-fenestrated Fontan procedure demonstrate an unobstructed Fontan circuit. There is no conduit stenosis and flow to the branch pulmonary arteries is unobstructed. Courtesy of Renelle George, MD (AngioAtlas.org)
Note how the interventricular septum bows into the left ventricle in systole.
Note connection and flow into the aorta from the left main system.
Right anterior oblique projection of the same right ventricular angiogram showing extensive right ventricle to coronary artery fistulae. The coronary circulation is bizarre and flow to the left ventricle appears tenuous.
This is the same patient shown in Video 16-12B.
This is the same patient shown in Video 16-11.
The tip of the catheter is in the high superior vena cava. Contrast injection demonstrates a patent bidirectional cavopulmonary (Glenn) anastomosis Fontan conduit. There is mild hypoplasia of the pulmonary artery to the left of the Glenn anastomosis.
Hand injection through the right internal jugular venous sheath in a patient with double outlet right ventricle, hypoplastic left ventricle, and bilateral superior vena cavae status post Glenn procedure demonstrates a widely patent right Glenn circuit. Courtesy of Renelle George, MD (AngioAtlas.org)
Stored fluoroscopy during deployment and locking of a 25mm Gore Cardioform Septal Occluder across the atrial septum of a patient with a fenestrated atrial septal defect. The left atrial disc is deployed first, and is pulled toward the right atrium before the right atrial disc is deployed. Courtesy of Renelle George, MD (AngioAtlas.org)
Stored fluoroscopy during release of a 25 mm Gore Cardioform Septal Occluder across the atrial septum of a patient with a fenestrated atrial septal defect. The device is in good position following release. Courtesy of Renelle George, MD (AngioAtlas.org)
This boy with hemiplegia has an equinus gait and upper extremity posturing often seen when walking and running.
Color Doppler demonstrates branch pulmonary arteries after the LeCompte maneuver.
Straight AP frontal plane view of right ventricular injection shows the infundibular stenosis and thickened pulmonary valve leaflets, in addition to the pulmonary artery bifurcation.
90-degree straight lateral projection again shows right ventricle well in addition to the right-to-left shunt through the VSD with filling of the left ventricle.
Note the catheter in the right ventricular infundibulum is directed towards the main pulmonary artery and is apposed to the valve tissue.
This is the same patient shown in Video 16-12A.
This is a newborn who had undergone radiofrequency perforation of an atretic pulmonary valve. Saturations were considered to be unacceptably low when prostaglandin was discontinued, and the duct nearly closed. Notice the pulmonary insufficiency into the relatively small right ventricular chamber. Also note the tubular, horizontal PDA anatomy. There is moderate constriction of the ductus near the pulmonary end.
The PDA stent was deployed and remained in excellent position. The stent was delivered under relatively low pressure and the small constriction in the stent was purposely not eliminated in this situation. As expected, the right ventricle gradually became more compliant, and saturations increased over time. On follow-up after 3 years, the patient was fully saturated and the ductus was still patent but with a relatively small volume of flow. Our routine is to close the PDA electively at around 6 years of age in these patients if the stented ductus has not closed spontaneously.
There was only a slight residual gradient into the left lower lobe.
The proximal left pulmonary artery has a tapering shape to the region of stenosis. The shape of the proximal pulmonary artery and proximity of the branching to the primary stenosis makes placement of a stent at this time, in this patient, less attractive than angioplasty.
A pigtail catheter is positioned in the body of the left ventricle from a retrograde approach and the cameras are angled to elongate the ventricular septum, which can help to visualize the aortic outflow tract and also evaluate for ventricular septal defects along the entire length of the ventricular septum. With injection, there is a distinct ridge of tissue below the aortic valve, consistent with a subaortic membrane and discrete subaortic stenosis.
NCLV and tricuspid atresia.
A large VSD with cephalic infundibular deviation and pulmonar stenosis (TOF variant) and NCLV.
NCLV and apical ventricular septal defect.
In the thumb, the MCP joint is stabilized by the volar plate when fully extended, so the MCP joint should be flexed 30° to assess the collateral ligaments. In the fingers, the collateral ligaments are slack in MCP extension and taut in MCP flexion, so the MCP joint should be flexed 90° before applying radial or ulnar deviation stress to assess the collateral ligaments.
The right atrium, tricuspid valve annulus, and right ventricle are larger than the left atrium, mitral valve annulus, and left ventricle.
A 10-year-old male basketball player with a right ankle injury and subsequent RSD. This video demonstrates mirror therapy utilizing his unaffected left lower extremity.
The branching pulmonary artery is seen arising from the left ventricle (inferior part of image), and the non-branching aorta is seen arising from the right ventricle (superior part of image). The atrioventricular valves are also well seen, and it is clear that the tricuspid valve has septal attachments, while the mitral valve does not.
This patient had severe tricuspid regurgitation and a large central gap in leaflet coaptation. The tricuspid leaflets were thickened and the chordae were shorter than normal (red arrow), this restricted the motion of all three leaflets. Despite the restricted mobility, these leaflets are not adherent to the underlying myocardium and the apical displacement index, representing the offset of the mitral and tricuspid valve septal insertions (white arrow), was only 6 mm/m2. This was not a case of Ebstein anomaly. L, left; LA, left atrium; LV, left ventricle; RA, right atrium; RV, right ventricle; S, superior.
(From Eidem BW, O'Leary PW, Cetta F. Echocardiography in Pediatric and Adult Congenital Heart Disease. 2nd ed. Philadelphia, PA: Wolters Kluwer; 2015.)
During the sweep, we see the left ventricular outflow tract and aorta, right ventricular outflow tract and pulmonary artery, the ductus arteriosus and the transverse aortic arch. In the most cephalad portion of the sweep, the view is that of the three vessel and trachea view; the transverse arch can be seen crossing leftward of the trachea, confirming a left aortic arch.
Note the laminar flow across the atrioventricular valves, and trivial tricuspid valve (right side of image) regurgitation.
More mild tightness can lead to hip and knee pain in normal athletes.
(Courtesy of Dr. Joseph Dearani and Mayo Foundation for Medical Education and Research.)
The heart is levocardic. The left ventricle is right-sided (top part of image) and is mildly dilated. The right ventricle is hypoplastic and left-sided (bottom of image). The branching pulmonary artery arises from the eft ventricle, and the hypoplastic aorta arises from the right ventricle, anteriorly and leftward of the pulmonary artery. A ventricular septal defect is briefly seen just under the pulmonary valve annulus.
There is a small PDA (red color Doppler flow) along with a mobile vegetation noted in the main pulmonary artery. Echo image courtesy of www.pedecho.org
There is diastolic right ventricle collapse consistent with tamponade physiology.
Thickened aortic valve with vegetation. In addition, there is a periaortic abscess noted. Echo image courtesy of www.pedecho.org
There is a large perimembranous outlet ventricular septal defect. There is left ventricular outflow tract narrowing with a hypoplastic pulmonary valve which arises from the left ventricle
The pulmonary artey is seen arising from the left verntricle (bifurcating vessel) and the aorta arising anteriorly from the right ventricle. Echo image courtesy of www.pedecho.org
There is a perimembranous outlet ventricular septal defect. The truncal valve overrides the VSD and appears dysplastic with thickened leaflets which demonstrate restricted excursion.
The tumor protrudes through the mitral valve in diastole.
The VSD is largely occluded by prolapsed aortic cusp. There is mild aortic regurgitation secondary to aortic valve distortion in the setting of aortic cusp prolapse
There is a large perimembranous outlet ventricular septal defect with aortic override noted from this view. Echo image courtesy of www.pedecho.org
There is a large perimembranous outlet ventricular septal defect with bidirectional shunting with aortic override noted from this view. Echo image courtesy of www.pedecho.org
Note the large pericardial effusion with the heart swinging in a to-and-fro fashion. The lateral and posterior aspects of the effusion are relatively stable in size throughout the cardiac cycle, while the anterior effusion is more apparent in systole.
It is suggested not to measure or calculate the NC/C ratio from this projection due to the possible oblique angle of the structures.
There is mild pulmonary regurgitation.
The aorta is positioned anterior and rightward to the pulmonary artery. Echo image courtesy of www.pedecho.org
There is a large outlet perimembranous VSD with anterior and superior deviation of the infundibular septum causing dynamic right ventricular outflow tract narrowing in systole. Echo image courtesy of www.pedecho.org
The pulmonary valve annulus is hypoplastic with a deficiency of valvar tissue. The branch pulmonary arteries are severely dilated and aneurysmal in appearance. Echo image courtesy of www.pedecho.org
An intraluminal thrombus is also noted.
There is flow turbulence across the pulmonary valve with severe regurgitation. Echo image courtesy of www.pedecho.org
Cross-section of the valve demonstrates very thickened, deformed leaflets with no distinct edges.
Longitudinal view of the valve again shows thickened, dysplastic leaflets and supra-annular narrowing. Notice the lack of doming seen in a typical stenotic valve.
The right side is short causing the pelvis to tilt downwards on that side. Notice how the pelvis tilts back and forth with each stride.
Cineangiogram via a pigtail catheter advanced retrograde to the ascending aorta demonstrates an unobstructed left aortic arch. There is a moderate-to-large Type C patent ductus arteriosus. Courtesy of Renelle George, MD (AngioAtlas.org)
Hand injection in the left subclavian artery demonstrates a moderate PDA arising from the underside of the aorta and taking a 270-degree turn before inserting into the left pulmonary artery. Courtesy of Renelle George, MD (AngioAtlas.org)
Hand injection through the axillary artery sheath following implantation of two 3.5 mm Integrity stents demonstrates a widely patent stented PDA with appropriate stent overlap on both aortic and pulmonic ends. Courtesy of Renelle George, MD (AngioAtlas.org)
Note the aortic root injection performed with a fully inflated balloon across the conduit to exclude coronary artery compression at the time of valve implantation. Recoil was observed during balloon sizing and therefore prestenting of the homograft was performed before valve insertion. Angiography in the pulmonary artery following valve implantation shows trivial pulmonary insufficiency. A 7 mm Hg gradient was measured across the Melody valve.
Diagram and video depicting tricuspid valve-in-valve placement from a femoral venous approach. In certain patients a right internal jugular vein approach may be preferred. With either technique, the stiff exchange wire is anchored in a distal pulmonary artery to facilitate movement and secure positioning of the delivery system across the tricuspid bioprosthesis. The Melody (MedtronicTM) and Sapien (EdwardsTM) percutaneous valve systems have been used in the United States in an “off -label” manner for this purpose. International experience with both systems has shown good results, obviating the need for surgery in many patients. From McElhinney DB, Cabalka AK, Aboulhosn JA, et al. Transcatheter tricuspid valve-in-valve implantation for the treatment of dysfunctional surgical bioprosthetic valves: an international multicenter registry study. Circulation. 2016;133(16):1582--1593.
The left ventricle now appears severely hypoplastic, and there is significant endocardial fibroelastosis.
This type of stent can be serially dilated to as much as 20 mm.
The catheter tip is placed in the right pulmonary artery with a pulmonary arteriovenous malformation demonstrated upon contrast injection.
Tortuous PDA with left to right flow suppling c onfluent branch pulmonary arteries in a patient with pulmonary atresia with intact ventricular septum
Suprasternal notch view of the aortic arch demonstrating a tortuous "curley cue" PDA arising from the underline of the aortic arch in a patient with pulmonary atresia with intact ventricular septum
Apical 4 chamber sweep with color Doppler demonstrating multiple rignt ventricle fistulae in a patient with pulmonary atresia with intract ventricular septum
Apical 4 chamber view demonstrating hypoplastic muscle bound right ventricle and hypoplastic tricuspid valve in a patient with pulmonary atresia with intact ventricular sepum
Cineangiogram through a Berman angiocatheter in the right ventricle of a patient with valvar pulmonary stenosis demonstrates a thickened pulmonary valve. The leaflets are doming with restricted mobility. Courtesy of Renelle George, MD (AngioAtlas.org)
The LAO projection demonstrates the LV outflow tract and membranous septum below the aortic valve.
Note the markedly thickened valve leaflets, supravalvar narrowing, and borderline hypoplastic pulmonary artery branches.
Note the relatively thin valve leaflets, dilation of the main pulmonary artery, and normal sized distal pulmonary artery branches.
The anterior/cranial view shows a mildly hypoplastic, heavily trabeculated RV and regurgitant tricuspid valve.
The same findings are demonstrated in the corresponding lateral view. There is a faint, narrow jet of contrast corresponding to the tiny pulmonary valve opening.
In the straight frontal plane view, the Sano shunt lies directly in front of the descending aorta, making it very difficult to see if there is stenosis in the Sano shunt.
Lateral view nicely demonstrates the position of the Sano shunt without evidence of stenosis in the Sano conduit itself (prominent right ventricular muscle bundles are present at origin). This view also shows the entire neoaortic arch very well.
Note proximal left pulmonary artery stenosis with a noncircular stenotic lumen. The projected angles appear in the upper right hand corner and allow selection of optimal angiographic views for intervention.
(Courtesy of Dan A. Zlotolow, MD.)
Lateral view demonstrates the dynamic infundibular narrowing well. There is significant poststenotic dilation of the main pulmonary artery.
Frontal plane view shows very dynamic infundibular stenosis with hyperdynamic RV function and prominent trabeculations. There is significant poststenotic dilation of the main pulmonary artery.
Same findings are demonstrated in the corresponding lateral view.
Right ventriculogram via a JR 2.5 catheter demonstrates a severely hypoplastic and hypertrophied right ventricle with no antegrade flow, consistent with pulmonary atresia. There is severe tricuspid valve regurgitation. Multiple right ventricular sinusoids are seen. Courtesy of Renelle George, MD (AngioAtlas.org)
Selective left main coronary artery angiogram via a JL 2 catheter demonstrates patent left circumflex and left anterior descending arteries. The LAD has multiple sinusoidal connections to the right ventricle. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram through a Berman catheter advanced to the anomalous right lower pulmonary vein (Scimitar vein) via the inferior vena cava demonstrates a large, unobstructed vessel. It enters the inferior vena cava at the same level as the hepatic veins, and narrows as it enters. Courtesy of Renelle George, MD (AngioAtlas.org)
The patient has a right aortic arch.
The patient has a left aortic arch.
She had leukemia and a central venous access device. Note the large circumferential pericardial effusion with the heart swinging
At least two pulmonary veins are seen draining into a confluence which subsequently drains into the coronary sinus. Echo image courtesy of www.pedecho.org
The pulmonary artery is noted to arise from the left verntricle and the aorta arises anteriorly at the end of the sweep from the right ventricle. Echo image courtesy of www.pedecho.org
Flow turbulence is demonstrated at the insertion site in the hepatic vein within the liver consistent with obstruction. Echo image courtesy of www.pedecho.org
There is a large perimembranous outlet ventricular septal defect. There is left ventricular outflow tract narrowing with posterior malalignment of the outlet septum with a hypoplastic pulmonary valve which arises from the left ventricle. A small PDA is seen with left to right shunting
The anomalous muscle bundle can be seen by 2D and obstruction confirmed by the mosaic pattern of the color Doppler through that region. There was severe obstruction with a maximum velocity of 5.2 m/s.
The tricuspid valve appears atretic with a plate-like oriface. There is a small to moderate sized ASD.
There appears to be a short main pulmonary artery segment giving rise to confluent branch pulmonary arteries which arise from the ascending aorta
The ECG and stress test are normal.
Aortopulmonary collaterals supplying segments to the right and left lungs have been unifocalized and the patient has undergone balloon angioplasties of both central branch pulmonary arteries and the right-sided MAPCA which supplies upper and lower lobe segments. Placement of an RV to PA conduit with a bioprosthetic valve was accomplished, along with VSD closure using a fenestrated patch. Forty to 50 mm Hg gradients persist into both branch pulmonary arteries. Note the small inferior aneurysm of the right pulmonary artery which is unchanged from angioplasty with an ultra--high-pressure balloon approximately 1 year previously.
Her foot is in equinus to accommodate the difference in length as a result of her dislocation. This is an example of a functional limb length discrepancy. Her femur is not anatomically short, but she has a joint problem (hip dislocation) that makes her functional limb short.
Note how short the chordae are, as well as the relative immobility of the mitral valve leaflets.
The upper left clip shows the relatively immobile mural leaflet, which can also be appreciated in the lower right three-dimensional image.
Clinical palpation and an MRI confirmed a primary neuroma of her peroneal nerve.
AO, aorta; ANT, anterior; LEAF, leaflet; POST, posterior.
Note the fusion of the chords and the restrictive orifice.
AL = aortic leaflet, AO = aorta, ML = mural leaflet, RV = right ventricle
Note how the inflow jet forms a funnel.
When asked to selectively dorsiflex the ankle, the child is unable. When he flexes the hip, a mass action pattern is activated, and the tibialis anterior fires, dorsflexing the ankle. (Video with permission of the Children's Orthopaedic Center, Los Angeles.)
The transverse arch and aortic isthmus are hypoplastic, and there is increased angulation of the arch. By color Doppler imaging, there is retrograde flow in the distal transverse arch.
A dilated aorta is seen overriding the ventricular septum and large ventricular septal defect. Cephalad (to the superior right of the image), the hypoplastic pulmonary artery with a hypoplastic pulmonary annulus is seen arising from the right ventricle.
The aorta is seen overriding the ventricular septum and large ventricular septal defect that has bidirectional flow. As we sweep cephalad, the hypoplastic pulmonary artery with a hypoplastic pulmonary annulus is seen arising from the right ventricle.
The four-chamber view appears relatively normal. As we sweep cephalad, a large ventricular septal defect is visible with an overriding dilated aorta. A bifurcating pulmonary artery is then seen, with flow towards the lungs from the branch pulmonary arteries. However, there is no connection between the right ventricle and the main pulmonary artery stump. Sweeping more cephalad, the transverse aortic arch is seen crossing to the right of the trachea, defining a right aortic arch. The first vessel arising from the aorta is the left innominate artery, which is directed to the left side of the fetus, which gives rise to the left subclavian artery, which then gives rise to the ductus arteriosus, which connects with the main pulmonary artery stump.
Color Doppler demonstrates moderate tricuspid regurgitation.
Both atrioventricular valves are well-developed and open into the left ventricle. The atrial septum is bowing into the left atrium.
The larger pulmonary artery arises from the left ventricle, and the mildly hypoplastic aorta arises from the hypoplastic anterior right ventricle. Blood flow into the right ventricle is solely via the ventricular septal defect.
At the level of the ascending aorta, the large aortopulmonary window, connecting the ascending aorta to the main pulmonary artery, is seen. Distal to the window the aorta is hypoplastic, and the aorta branches into three head and neck vessels, with no apparent transverse arch.
There has been growth arrest of the left ventricle, while the right ventricle has continued to grow, now making the left ventricle mildly hypoplastic. There continues to be severe left ventricular dysfunction and endocardial fibroelastosis.
In this example, no left ventricular chamber is visible, and there is a small left atrium with a prominent left atrial appendage. The right atrium and right ventricle are dilated.
Note that there is a very narrow but apex-forming left ventricle, and mitral annular hypoplasia. Not seen in this image are moderate hypoplasia of the aortic valve annulus and severe aortic arch hypoplasia.
There is severe left ventricular dysfunction, a dilated and globular left ventricle, and endocardial fibroelastosis. The aortic valve annulus is hypoplastic, and the valve leaflet motion is very restricted.
The distal end of the ductal arch can be seen entering into the descending aorta, and it appears very large in comparison with the distal arch and isthmus.
Color Doppler shows ductal flow entering the main pulmonary artery (red) and bouncing off the valve (blue) with no clear forward flow, but a tiny yet was seen angiographically.
The tumor is predominantly inferior to the left ventricle extending into the ventricular septum, splaying apart the ventricular chambers. There is also an anterior pericardial effusion.
There is left ventricle-right ventricle size discrepancy. Although the left ventricle appears mildly hypoplastic, the left ventricle is apex-forming. Sweeping cephalad, the ascending aorta and transverse arch are much narrower than the main pulmonary artery and ductus arteriosus. There is also a left superior vena cava best seen in the more cephalad views.
The four-chamber view appears normal. Then, as we sweep cephalad, a single great artery (the truncus) is seen overriding the ventricular septum. Four vessels ultimately arise from this trunk: first, a hypoplastic ascending aorta, then branch pulmonary arteries, and finally a large ductus arteriosus is seen connecting to the descending aorta. While the ductus arteriosus is usually absent in truncus arteriosus, it is present and large in this case since the aortic arch is interrupted.
The four-chamber view appears relatively normal, although there is cardiomegaly. As we sweep cephalad, the left ventricular outflow tract appear normal. Just before the right ventricular outflow tract comes into view, a large connection between the ascending aorta and another structure is seen, which becomes the main pulmonary artery. This connection is the aortopulmonary window.
In the four-chamber view, a large primum atrial septal defect, common atrioventricular valve, and large ventricular septal defect are visible. As we sweep cephalad, the ventricular septal defect continues to be visible and an overriding dilated aorta is seen. Confluent branch pulmonary arteries are then seen, although there is no connection between these and the right ventricle. Another tortuous vessel (the ductus arteriosus) is visible that extends from the bifurcation of the branch pulmonary arteries to the aorta.
Visible are bilateral left atrial appendages, a complete common atrioventricular septal defect with the valve opening predominantly into the right ventricle, a large ventricular septal defect that extends from the inlet to the outlet septum, pulmonary annulus hypoplasia, and a left aortic arch. Also visible are a large hemiazygous vein posterior to the aorta and a single left-sided superior vena cava.
Although the right ventricle is hypoplastic, the pulmonary artery is well-developed, likely due to flow across the ventricular septal defect.
Ultrasound can be used as a diagnostic study and/or as an adjunct to physical examination.
This boy with spastic hemiplegia has classic findings in the upper extremity including flexion, ulnar deviation, and decreased supination. He has difficulty with grip and is only able to actively extend his fingers with the wrist flexed.
This adolescent girl with MFS has genu valgum which is not symmetric and affects her ambulatory ability.
Please note skin dimpling and medial-sided ray duplication commonly seen with tibial deficiency.
Cineangiogram via a pigtail catheter advanced retrograde across the aortic arch and into the left ventricle of a patient with a perimembranous ventricular septal defect demonstrates a mildly dilated left ventricle with qualitatively normal function. Contrast is seen entering the right ventricle through the large perimembranous ventricular septal defect. There is a large ventricular septal aneurysm which partially closes the defect. Courtesy of Renelle George, MD (AngioAtlas.org)
Cineangiogram via a pigtail catheter advanced retrograde across the aortic arch and into the left ventricle of a patient with a perimembranous ventricular septal defect demonstrates a mildly dilated left ventricle with qualitatively normal function. Contrast is seen entering the right ventricle through the large perimembranous ventricular septal defect. There is a large ventricular septal aneurysm which partially closes the defect.Courtesy of Renelle George, MD (AngioAtlas.org)
A positive test is one that provokes dorsal wrist pain (even though no direct pressure is applied dorsally) or a palpable and painful clunk in the wrist when pressure is released.