Adult Congenital Heart Disease-What Every Cardiologist May See or Miss
Adult Congenital Heart Disease-What Every Cardiologist May See or Miss
Review\r
Management of adults with congenital heart disease and things not to miss in\r
the evaluation and care of these patients.
Content
5.52 -> and why should you know about congenital
7.279 -> heart disease because there's about two
9.04 -> million of them now in north america so
11.599 -> you're going to be seeing them a lot in
13.36 -> your practice and i'm going to go
14.639 -> through a lot of cases and some common
17.039 -> things that we see that cardiologists
19.52 -> often overlook
21.439 -> and how you may avoid those mistakes
25.84 -> so just for those history buffs out
27.599 -> there i'd remind you that in 1801 lord
30.48 -> nelson put the telescope to his blind
32.8 -> eye
33.6 -> and said i see no ships and hopefully
36.8 -> you won't do this this gives rise to the
39.04 -> expression turning a blind eye
41.28 -> and when you're doing echoes hopefully
43.52 -> you won't be turning any blind eyes and
45.52 -> i'm going to go through then six
48.16 -> things that i hope you won't miss
50.399 -> in your practice and on your echo
52.719 -> imaging
54.239 -> so this is all going to be case-based
56.719 -> and i'm going to begin with case number
59.359 -> one and here's a mistake maybe we could
61.6 -> turn the lights down just a little
63.199 -> please
64.08 -> so let's begin with a simple lesion
66.24 -> bicuspid aortic valve as you know it's
68.72 -> one of the most common congenital
70.96 -> lesions but i want to remind you of the
72.96 -> genetic implications
75.04 -> and how important it is to screen first
78 -> degree relatives and of course you will
79.84 -> remember the autopathy that's associated
82.96 -> with this bicuspid valve the so-called
84.96 -> cystic medial necrosis
87.68 -> so here's a case a 36 year old man who
90.88 -> was transferred to our hospital
92.56 -> following svt
94.799 -> his father has a history of a dilated
97.119 -> aorta grandfather bicuspid aortic valve
100.799 -> and a co-oct and had surgery but neither
104.32 -> the patient nor his brothers had had
107.68 -> echo screening and i draw your attention
110.56 -> to the chest x-ray that shows not only a
113.36 -> big heart but also a really dilated
116.799 -> aorta
118.159 -> so now he's in with his episode of sbt
122.32 -> and here's his echocardiogram as you see
124.399 -> in this long axis
126.159 -> format his lv is huge both in systole
130.16 -> and diastole and you can see this aortic
132.879 -> valve that looks a bit lumpy and is
135.12 -> doming and in short axis you can see
137.92 -> that there's some fibrosis and
139.879 -> calcification and it's a bicuspid valve
142.72 -> with a raphae over here at the top
146.319 -> and
147.68 -> velocity 3.4 mean gradient 30 across
151.519 -> that valve ventricle not looking
153.92 -> entirely happy as well as dilated and so
157.04 -> as we turn our attention to the color
159.12 -> you can see there's important aortic
161.12 -> regurgitation look how pulsatile that
164.239 -> aorta is in the middle and as you see on
167.44 -> the right that red flow going back
170.4 -> and that holo diastolic reversal in the
172.959 -> descending aorta telling you that that's
175.36 -> severe aortic regurgitation
178.48 -> so it was the svt that brought him in
181.84 -> there's his aorta 49 millimeters so
185.519 -> approaching the time when surgery would
187.84 -> be indicated anyway for this valve and
190.8 -> so he needed a root replacement with a
192.879 -> mechanical aortic valve but of course
195.44 -> early on because that ventricle was so
197.599 -> big and not functioning well his early
200.239 -> post-op ef is impaired
202.959 -> and obviously it would have been great
205.04 -> to get him earlier than that you can see
207.44 -> how dilated this aorta was on imaging
211.44 -> so in the sense the svt saved him and
214.4 -> i'd remind you that the risk of
215.92 -> dissection with bicuspid valve is about
218.799 -> nine times higher than the general
220.799 -> population
222.159 -> and about nine percent of first degree
224.319 -> family members have a bicuspid valve in
226.959 -> one series almost a third
229.28 -> and another nine percent have some other
231.68 -> congenital heart disease so it's almost
234 -> 20 percent of first-degree relatives and
236.879 -> so really in the sense this svt for this
240.159 -> patient saved him before he had any
242.48 -> further catastrophe but still his ef
245.2 -> wasn't normal so screening first-degree
248.08 -> relatives with an echo is absolutely key
251.519 -> for your patients with bicuspid valve
254.799 -> let's go on to mistake number two here's
257.199 -> a 65 year old woman referred for a vsd
261.199 -> and possible
262.639 -> severe pulmonary hypertension that she'd
264.96 -> been diagnosed by echo at home and as
268.32 -> you start to look in the perimembranous
270.4 -> septum here you can start to see a
272.24 -> little bit of a drop out and her lv is
275.84 -> big 58 millimeters so is this a
279.04 -> significant vsd
281.6 -> so we need to look a bit further and
283.36 -> there again in the perimembranous region
285.6 -> you can see this left to right shunting
288.08 -> across the vsd
289.759 -> the rv is also slightly enlarged and
293.04 -> that vsd we're measuring here at
295.44 -> approximately at least six millimeters
298 -> in size so certainly not very small
301.759 -> so we start to look at our velocities
304.16 -> and here you can see flow through the
305.759 -> vsd both in systole and diastole and we
309.44 -> have a velocity of 4.7 meters a second
313.12 -> through the vsd
314.96 -> which would suggest that the rv pressure
317.68 -> was relatively low so not much pulmonary
320.88 -> hypertension
322.8 -> here's an apical format now we've got
324.96 -> apex down so the rv is over here and you
328.56 -> can see there's at least a modest amount
330.8 -> of tricuspid regurgitation and you can
333.28 -> also see flow through the vsd right here
337.199 -> and so
338.4 -> we start trying to get a signal of the
341.52 -> tr velocity and that's what they'd got
344.24 -> 4.3 meters a second so now they're
348.24 -> worried about pulmonary hypertension but
350.56 -> is that the velocity through the vsd
355.759 -> so we start to look again and here you
358.24 -> can see the septal leaflet of the
360 -> tricuspid valve is starting to get
362.639 -> tacked down over the vsd which it does
365.52 -> it forms an aneurysm of the membranous
367.759 -> septum that's how vsds close this one
370.96 -> obviously not completely closed
373.36 -> and again trying to get the tr velocity
375.919 -> from the rv inflow view and that's the
378.8 -> velocity that they took
380.72 -> which is about four meters a second
383.52 -> but you can't do that because the vsd
386.72 -> velocity
388.16 -> through that jet is getting all tangled
390.56 -> up with the tr velocity that is not a
393.52 -> clean
394.4 -> tr velocity
396.72 -> so
397.759 -> you turn your attention to the pulmonary
400.24 -> valve and look at the pulmonary
401.84 -> regurgitation and there even though it's
404.4 -> faint you can get an npr of 0.9 meters a
407.6 -> second
408.4 -> confirming that this patient actually
411.039 -> has no pulmonary hypertension whatsoever
414.72 -> so remember the vsd velocity was high
418.24 -> but do not get tangled up with
421.44 -> the vsd velocity and the tr because
423.919 -> they're so close together and that tr
426.479 -> jet is contaminated when the vsd is
429.84 -> close to the tricuspid valve so look at
432.56 -> other things check the pr velocity this
435.84 -> lady had a big lv and when the lv is big
439.12 -> with the vsd the vsd
441.44 -> should be closed which hers was and the
444.08 -> tricuspid valve had an annuloplasty
447.199 -> so another mistake don't confuse those
449.68 -> two jets and check the pr velocity
453.52 -> number three
455.919 -> this one i get very regularly although i
458.4 -> hammer on about it all the time causes
460.72 -> of right ventricular enlargement and
463.039 -> this is a common phone call i have a
465.199 -> patient with pulmonary stenosis and a
468.16 -> big rv
469.68 -> but the pulmonary velocity on echo is
472.24 -> only two and a half meters a second
475.039 -> what do i do
476.879 -> and the answer is you never get a big rv
481.28 -> with isolated pulmonary stenosis you get
484.16 -> hypertrophy
485.68 -> but you don't get a big rv
489.039 -> so why have you got a velocity of 2.5
492.56 -> meters a second
494.24 -> because of a shunt here you see an
496.4 -> example of an asd
498.639 -> and of course the answer is is that you
500.72 -> get increased flow as you see in that
502.96 -> white arrow out through the pulmonary
505.52 -> artery
506.479 -> simply because of the left to right
508.639 -> shunt
509.52 -> so you get that velocity of 2.5 meters a
512.64 -> second just because of the left to right
515.599 -> shunt it is not pulmonary stenosis
519.44 -> so you have to find a shunt when you see
522 -> the big rv and of course transthoracic
524.8 -> echo is very important and i'm going to
527.279 -> show you some ways to help you find them
529.76 -> but of course if you can't find it you
531.6 -> have to keep digging and you might need
533.44 -> a tee or some other imaging like mri
538 -> here's a classic chest x-ray of an asd
541.2 -> so you have to look everywhere look at
543.68 -> every window that's my message if it's
546.32 -> not a secundum look for sinus venosus
549.519 -> and if you can't find an asd anywhere
552.959 -> look for anomalous pulmonary venous
555.76 -> drainage which can happen as an isolated
558.959 -> lesion and make for a big rv
562.48 -> so explore every window and i'd
564.72 -> encourage you to look both subcostal as
567.36 -> well as suprasternal and i'd remind you
570.24 -> for example that the sinus venosus
572.48 -> defect which is much harder to find on
575.04 -> transthoracic echo
577.04 -> is posterior to the fossa ovalis as you
580.8 -> see here so that makes it harder to find
583.76 -> and it's in the superior portion of the
585.839 -> septum and it's also associated with
588.64 -> anomalies right upper pulmonary vein
590.72 -> almost always so it's a double volume
593.68 -> overload for the right ventricle so how
596.8 -> do you find these on transthoracic echo
600.56 -> so here's an example you can see in the
603.2 -> short axis format that lv is a bit
606 -> d-shaped and the right ventricle is
608.48 -> enlarged and this one is also
610.8 -> hypertrophied
613.2 -> here in the apex down format you can see
616 -> there's some tr
617.519 -> and the rv again is dilated and is
621.44 -> hypertrophied
623.44 -> and the tr velocity there's some
625.12 -> pulmonary hypertension here the tr even
627.6 -> though it's faint is four meters a
629.68 -> second so we're worried about
631.68 -> pulmonary hypertension so where is the
634.88 -> shunt
635.839 -> and this is when you want to really dig
637.76 -> in the subcostal window and this is how
640.48 -> you usually find them often getting the
642.32 -> patient to take a deep breath in and as
644.72 -> we're looking at the right atrium and
646.88 -> the atrial septum you can see there's a
649.279 -> big area of drop out here and this is
652.32 -> the really large sinus venosus asd that
656.32 -> we can measure at about 1.4 centimeters
660.399 -> so that's giving you the big rv and the
662.72 -> pulmonary hypertension and the subcostal
665.36 -> window is the way to go if you can't
667.92 -> find the defect again think about doing
670.72 -> other imaging like t-e-e or mri
676.16 -> okay mistake number four
679.2 -> never assume because a surgeon's been in
681.76 -> the chest before that the complete
684.399 -> diagnosis has been made and again i'd
686.64 -> encourage you to
688.48 -> always even if you think this is a
690.079 -> routine post-op study just do a really
693.279 -> comprehensive study because things may
695.6 -> not fit and things can be overlooked
698.56 -> because maybe the surgeon didn't find
701.2 -> absolutely everything
703.36 -> so here's another example
705.92 -> this is an x-ray of a 45 year old woman
709.279 -> who had a murmur heard when she was 21
712 -> and was found to have a secundum asd
715.04 -> and that had been closed by a sternotomy
717.92 -> many years before
720 -> and so you would think she should be
721.68 -> doing okay but in the last three years
723.839 -> her exercise capacity has been going
726.48 -> down
727.44 -> and on exam um sinus rhythm normal jvp
732.32 -> but the right ventricular parasternal
734.72 -> impulse is prominent and she's still got
738.72 -> two over six systolic ejection murmur at
741.92 -> the left stone ledge and you might say
744.399 -> on here that she
746.079 -> may have some increased pulmonary
748.16 -> plethora and perhaps some rv enlargement
751.44 -> as you see in this chest x-ray so let's
755.12 -> turn our attention to the echo has her
757.68 -> asd patch become dehyst what the heck is
760.72 -> going on why has she got the murmur and
763.04 -> why is she not doing well
765.6 -> so here's the short axis and
768.8 -> um you can see here that the right
770.639 -> ventricle is still quite large function
774.079 -> is good lv function is good and so we
777.36 -> have to figure it out has she got a
778.88 -> residual asd why hasn't the rv
782.639 -> remodeled favorably
785.6 -> so again we're zooming our attention on
788 -> to the atrial septum again this is apex
790.399 -> down
791.279 -> format
792.48 -> and in the area of the fossa virulus it
795.76 -> looks a bit redundant but we're
797.2 -> certainly not seeing any significant
799.519 -> color flow across where a secundum asd
803.04 -> patch has been so again why is the rv
807.2 -> enlarged
808.8 -> so we turn our attention to the
810.56 -> suprasternal window and here um you can
814.16 -> see the aorta on the left and in the
816.959 -> middle
817.92 -> as you start to look and over here on
819.839 -> the right
821.04 -> you can see there's some red flow here
823.279 -> lateral to the aorta so here's the aorta
826 -> in blue
827.12 -> but there is red flow heading north and
830.959 -> here we can delineate it a bit further
833.68 -> but there is something sizeable
836.32 -> way over here in the left chest that's
839.04 -> got red pulsatile flow heading north
842.639 -> so what can that be we put our doppler
845.44 -> signal in there
847.44 -> and we see again this low velocity
850.88 -> pulmonary flow or low velocity venous
853.92 -> flow heading
855.76 -> towards the head and as you see this is
858.639 -> going up
860 -> coming around and heading down towards
863.44 -> the heart
864.88 -> so what is this this is anomalous left
868.32 -> pulmony venous drainage and this is
870.88 -> drainage from the left lung
873.04 -> heading into a vertical vein which goes
875.839 -> into the innominate vein and round to
878.32 -> the svc
879.76 -> so this lady although she had her asd
882.399 -> closed has this huge amount of flow
885.12 -> coming from the left lung and of course
887.519 -> the surgeon wouldn't see that because
889.44 -> this is over on the side of the chest
892 -> while the surgeon was focusing in the
893.839 -> middle and of course this requires
896 -> surgical repair and here what you do is
898.399 -> you anastomose this anomalous venous
901.36 -> drainage the vertical vein to the left
904 -> atrial appendage through the left chest
906.56 -> and then you can resolve that rv volume
909.04 -> overload so again i show you this case
911.6 -> just to demonstrate that because a
913.76 -> surgeon's been in there things may be
915.839 -> left things may be unnoticed and again
918.48 -> importance of going through all these
920.24 -> windows suprasternal subcostal and
922.959 -> here's the answer that um this lesion
925.6 -> was overlooked and she required a second
928.48 -> surgery to get rid of this anomalous
931.279 -> venous drainage
934.079 -> okay mistake number five
937.839 -> here's a 30 year old woman referred for
940.32 -> evaluation of an abnormal chest x-ray
944.72 -> and here you can see her aorta is a
947.36 -> little bit enlarged four centimeters
950.079 -> she's a petite little body only about
952.48 -> five foot and weighs about 90 pounds
955.12 -> left ventricular function i think you'll
957.44 -> agree looks good
959.839 -> and um
961.199 -> she's also quite petite and we always
963.68 -> begin our imaging from subcostal and
965.92 -> we've got a nice view of this aortic
968.399 -> valve here would everybody agree this is
970.32 -> a three-cuspid
971.839 -> valve
974.56 -> so nice images
976.56 -> and
977.519 -> we look at everything from the abdomen
979.44 -> here's the aorta and we're looking at a
982.24 -> flow in the abdominal aorta from
984.88 -> subcostal we're actually getting good
987.04 -> images this too is from subcostal the
989.279 -> apical format we're looking at that
991.36 -> aortic valve again because we thought it
993.12 -> was a little bit thickened for a young
995.12 -> woman and you can see that there's just
997.12 -> a little bit of aortic regurgitation
1000.48 -> but again a good lv
1002.72 -> and there's the velocity across the
1004.24 -> aortic valve only 1.4 meters a second
1008.399 -> but we've seen that her ascending aorta
1010.48 -> is a bit dilated so we want to look at
1012.56 -> the rest of the aorta and so again
1015.279 -> suprasternal images are a bit
1017.6 -> challenging
1018.88 -> but we want to look down the descending
1021.12 -> aorta is there any co-octation and
1024.16 -> here's the velocity down the descending
1026.079 -> aorta
1027.199 -> one meter per second and we don't see
1029.76 -> evidence of turbulent flow
1032.72 -> so i'd ask you maybe we can have the
1034.88 -> lights up a little bit what's the
1036.24 -> diagnosis i've shown you everything you
1038.079 -> need to know
1039.439 -> so the clue was
1041.839 -> first of all here's a chest x-ray and
1044 -> you can actually see a figure 3 sign
1046.559 -> here and some rib notching
1049.2 -> but nobody of course had ever felt her
1051.44 -> femoral pulses
1053.28 -> and here's her abdominal aortic doppler
1056.88 -> and
1058.24 -> the aorta is small but look at this flow
1061.6 -> it shouldn't look like that you've got a
1063.28 -> very slow up stroke
1065.28 -> and a slow decay
1067.679 -> and a normal abdominal aorta flow should
1070.4 -> look like this right brisk up stroke
1072.72 -> rapid deceleration
1074.64 -> and hers instead look rather like that
1077.2 -> like a wrigley worm and the problem is
1079.919 -> it's severe co-octation
1082.32 -> but the mistake is i showed you a
1084.96 -> descending aortic velocity right and it
1087.679 -> was one meter a second
1090.16 -> it's great when it looks like this at
1092.08 -> four meters a second for a severe
1094 -> co-octation
1095.52 -> but the challenge is if you have a
1097.679 -> co-octation that's like this that's so
1100.72 -> severe there's barely a red cell
1102.559 -> squeaking through there when you're
1104.64 -> doing your suprasternal doppler
1107.28 -> and trying to get down that little
1109.28 -> narrowing echo can miss that very easily
1113.28 -> if the doppler is not absolutely aligned
1116.64 -> and that's what happened in her her
1118.4 -> co-octation was about one millimeter
1121.2 -> across very very severe and so
1124.559 -> suprasternal misses it you absolutely
1127.52 -> have to do abdominal flows always
1130.64 -> otherwise you miss that diagnosis
1134.16 -> so
1135.039 -> any time you suspect it think about
1137.919 -> other imaging and always remember your
1140.72 -> abdominal aortic doppler flow
1144 -> of course it would be great if somebody
1146.08 -> had felt her femorals she had
1147.919 -> hypertension which was very severe which
1150.559 -> had gone completely unnoticed very
1153.039 -> severe hypertension on four drugs nobody
1155.679 -> ever felt her femoral pulses and her
1157.6 -> feet were always freezing
1159.36 -> so the message from the echo perspective
1162.48 -> always think about the subcostal
1164.24 -> abdominal aorta because the descending
1166.4 -> aorta may miss it and think about doing
1169.12 -> other imaging like mr
1171.84 -> or ct
1175.12 -> okay the last one mistake number six
1179.2 -> and this is um
1181.36 -> i know how difficult it is to know about
1183.679 -> all the residual and sequelae of
1186.24 -> surgical repairs particularly when
1188.32 -> they're complex and of course patients
1190.64 -> who have complex congenital heart
1192.48 -> disease should be followed in special
1194.72 -> congenital heart disease centers but
1196.72 -> many are lost and you'll be seeing i'm
1199.36 -> sure if you haven't already seen a lot
1201.12 -> in your practice
1202.559 -> so let's focus on this mistake and this
1205.52 -> is of course um one of the most common
1208.559 -> cyanotic heart lesions tetralogy of
1211.12 -> fallow and probably taking you back to
1213.039 -> medical school i'd remind you that
1214.64 -> there's this big vsd
1216.72 -> an astronautic lump of muscle between
1219.2 -> the rv outflow tract sometimes also with
1222.559 -> a stenotic pulmonary valve and so
1225.2 -> patience is cyanotic because blue blood
1227.6 -> can't easily get out the pulmonary
1229.36 -> artery goes through the vsd to take the
1232.24 -> path of least resistance and into the
1234.88 -> aorta
1236 -> to make them blue
1238.159 -> and we've been repairing this lesion
1240 -> since the 1950s and typically the
1242.88 -> surgeon will close the vsd and resect
1246.159 -> that muscle so the blood can easily get
1248.88 -> to the pulmonary artery but of course
1251.2 -> since the pulmonary artery and the
1253.36 -> outflow of the rv is usually very small
1256.72 -> frequently the surgeon will have to
1258.799 -> patch the rv outflow to make sure blood
1262 -> can get out the pulmonary artery and
1264.48 -> that patch may frequently extend all the
1267.2 -> way across the annulus
1269.6 -> and out to the pulmonary artery itself
1272.88 -> um but here's the case a 38 year old man
1276 -> who had repair of tetralogy at age three
1279.679 -> and look at this chest x-ray and the
1281.84 -> first teaching point is
1284.159 -> if the repair is good the heart size
1286.24 -> should be normal
1287.919 -> obviously it's not this is what his
1290.72 -> doctor wrote he was fixated on the rv
1293.6 -> enlargement and decreased function i
1295.76 -> explained it's reassuring he hasn't
1297.52 -> developed pulmonary hypertension and
1300.48 -> that the rv can continue to function
1302.559 -> fairly well as it doesn't have to
1304.08 -> generate significantly high pressures
1306.32 -> but he's not feeling well
1308.159 -> if he continues to have decreased rv
1310.559 -> function he may progress to rv assist
1313.44 -> device and potentially a transplant
1316.88 -> and the patient read this and referred
1319.2 -> himself
1320.72 -> his is echo
1322.4 -> you can see his rv as we thought from
1324.72 -> the chest x-ray is big
1327.12 -> and his images are difficult because he
1329.12 -> weighed about 280 pounds so we're trying
1332.159 -> to look at the outflow here and the
1334.32 -> pulmonary valve but the tr velocity was
1337.36 -> only 2.4 so the rv pressure was around
1340.88 -> 30 so we're not thinking important
1343.44 -> pulmonary stenosis
1345.52 -> so let's look at this outflow here's the
1347.84 -> aorta in the middle the pulmonary valve
1350.88 -> what's left of it and there you see this
1353.52 -> to and fro flow
1355.76 -> and here's the doppler
1358.4 -> one meter a second moving forwards
1361.919 -> so the diagnosis is
1364.64 -> shout out
1367.52 -> wide open pulmonary regurgitation easy
1370 -> to miss
1371.12 -> red flow going backwards the doppler's
1374 -> the clue
1375.28 -> no stenosis with forward velocity but
1377.76 -> look at this rapid deceleration back
1380.799 -> down to baseline this is severe
1383.36 -> pulmonary regurgitation this is why the
1385.919 -> rv is big and when it's this big there's
1388.96 -> a higher risk of vt and sudden death
1391.919 -> murmur of pr overlooked completely
1394.88 -> overlooked on echo this person couldn't
1397.28 -> get pulmonary hypertension he was born
1399.28 -> with pulmonary stenosis
1401.2 -> so the doctor
1402.559 -> overlooked everything overlooked the
1404.72 -> echo and what this man needed was a
1407.44 -> pulmonary valve replacement before the
1410.32 -> rv was shot
1412.159 -> so my take-home points in the last
1414.559 -> minute bicuspid valve remember to screen
1417.76 -> the first degree relatives
1420.08 -> vsd if the lv is big it should be closed
1423.52 -> and never confuse the tr jet with the
1426.32 -> vsd because you'll overcall pulmonary
1428.96 -> hypertension
1430.4 -> if there's a big right heart find the
1432.48 -> shunt
1433.6 -> don't assume the surgeon has made all
1435.679 -> the diagnoses at the time of surgery and
1438.559 -> beware co-octation is easy to miss
1441.039 -> remember the abdominal doppler think
1443.36 -> about alternative imaging and tetralogy
1446.64 -> of fellow if your patients
1448.72 -> got a good repair heart size should be
1450.64 -> normal and if the rv is big always look
1454.48 -> for pulmonary regurgitation so
1456.88 -> comprehensive echoes are important no
1459.76 -> matter what keep your eye on the prize
1462.64 -> don't get distracted you may miss
1465.36 -> something big thanks very much for your
1467.279 -> attention
1475.039 -> you
Source: https://www.youtube.com/watch?v=GLa-ph24s_4