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B?nh tim b?m sinh ? tu?i l?n (Adult congenital heart disease)

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B?nh tim b?m sinh ? tu?i l?n
(Adult congenital heart disease)

1. ??i c??ng.

1.1. ??nh ngh?a:
B?nh tim b?m sinh l s? b?t th??ng v? c?u trc tim v m?ch mu l?n ngay t? khi tr? ???c sinh ra.
B?nh chi?m t? l? 0,3-1% tr? s? sinh. C nhi?u b?nh ph?c t?p lm cho tr? t? vong s?m ngay sau khi sinh; m?t s? t?n t?i ???c ??n tu?i tr??ng thnh.
1.2. Tm t?t s? hnh thnh h? th?ng tim m?ch v tu?n hon ? thai nhi bnh th??ng:
- H? tu?n hon ???c hnh thnh t? l phi ngoi t? ngy th? 25 c?a phi thai, ??n tu?n th? 8-12 th c? quan tu?n hon ???c hnh thnh ??y ?? v hon ch?nh. ?y l giai ?o?n hay gy ra b?nh tim b?m sinh nh?t ??i v?i thai nhi.
- ? phi thai, sau khi h? tu?n hon hnh thnh, mu t?nh m?ch ch? d??i ???c nh?n t? t?nh m?ch r?n ?? v? nh? ph?i r?i chia ra hai ???ng: 60% mu xu?ng th?t ph?i ln ??ng m?ch ph?i, nh?ng do thai nhi ch?a th?, s?c khng c?a ph?i cao nn mu ph?i qua ?ng ??ng m?ch (?ng Botal) ??n ??ng m?ch ch? ?i nui c? th?; 40% mu cn l?i ? nh? ph?i ?i qua l? b?u d?c c?a vch lin nh? sang nh? tri r?i xu?ng th?t tri, qua van ??ng m?ch ch? vo h? th?ng ??ng m?ch ?i nui c? th?.
Sau ?? 1-4 tu?n th l? b?u d?c v ?ng Botal t? ???c ?ng l?i. V m?t l do no ? m l? b?u d?c v ?ng Botal khng ?ng ???c th tr? s? b? thng lin nh? ho?c t?n t?i ?ng ??ng m?ch.
1.3. Nguyn nhn:
Nguyn nhn b?nh ch?a ???c r rng nh?ng ng??i ta nh?n th?y c nhi?u tc nhn gy nn, bao g?m 2 nhm chnh l do y?u t? mi tr??ng v di truy?n; th??ng l cc y?u t? ny ph?i h?p v?i nhau:
- Nhi?m virut (nh?t l cm, rubeol), nhi?m khu?n.
- Ho ch?t (ch?t ??c mu da cam...).
- Tia x?, tia X.
- Y?u t? di truy?n qua gen.
- Do thu?c: khng sinh, hormon, thu?c ch?ng ??ng kinh, thu?c ch?ng ung th?, thu?c ?c ch? mi?n d?ch, sulfamid...
2. Phn lo?i b?nh tim b?m sinh (theo Perloff J.K.).
Phn lo?i c th? d?a vo lm sng hay gi?i ph?u h?c ho?c phi thai. Phn lo?i lm sng th??ng ???c s? d?ng v thu?n ti?n trong ch?n ?on v gi?ng d?y.
2.1. T?t b?m sinh chung c?a tim:
- V? tr b?t th??ng c?a tim.
- Bl?c nh?-th?t hon ton b?m sinh.
- B?t t??ng h?p nh?-th?t v th?t-??i ??ng m?ch (atrioventricular and ventriculoarterial discordance).
2.2. B?nh tim b?m sinh khng tm, khng dng ch?y thng (shunt):
2.2.1. B?t th??ng b?t ngu?n t? bn tri c?a tim (t? g?n nh?t ??n xa nh?t):
+ T?c ngh?n ???ng vo nh? tri:
- H?p t?nh m?ch ph?i.
- H?p l? van 2 l.
- Tim ba bu?ng nh? (Cor-triatristum).
+ H? van 2 l:
- Knh nh?-th?t [thng sn nh?-th?t (atrio ventricular canal)].
- B?t t??ng h?p nh?-th?t v th?t-??i ??ng m?ch (atrioventricular and ventriculoarterial discordance).
- ??ng m?ch vnh tri b?t ngu?n t? ??ng m?ch ph?i.
- M?t s? d? t?t khc [th?ng van tim b?m sinh, thi?u dy ch?ng, l van sau ch? ?i (cleft of posterior mitral valve), dy ch?ng ng?n b?t th??ng].
+ X? chun gin ho n?i m?c tim nguyn pht (primary endocardial fibroelastosis).
+ H?p ??ng m?ch ch?:
- H?p d??i van.
- H?p t?i van.
- H?p trn van.
+ H? van ??ng m?ch ch?.
+ H?p eo ??ng m?ch ch? (coarctation of the aorta).
2.2.2. B?t th??ng b?t ngu?n t? tim bn ph?i (t? g?n nh?t ??n xa nh?t):
+ B?nh Ebstein.
+ H?p ??ng m?ch ph?i:
- H?p d??i ph?u.
- H?p t?i ph?u.
- H?p t?i van.
- H?p trn van (h?p thn ??ng m?ch ph?i v nhnh).
+ H? van ??ng m?ch ph?i b?m sinh.
+ Gin thn ??ng m?ch ph?i v c?n.
+ T?ng p ??ng m?ch ph?i nguyn pht.
2.3. B?nh tim b?m sinh khng tm, c dng ch?y thng:
2.3.1. Dng ch?y thng ? t?ng nh?:
+ Thng lin nh? (atrial septal defect - ASD)
- L? thng tin pht (ostrium prium).
- L? thng th? pht (ostium secundum)
- Xoang t?nh m?ch (sinus venosus).
- Xoang vnh (coronary sinus).
+ N?i li?n b?t th??ng t?nh m?ch ph?i bn ph?n (t?nh m?ch ph?i v? l?c ch? bn ph?n: partial anomalous pulmonary venous connections).
+ Thng lin nh? km h?p l? van 2 l (h?i ch?ng lutembacher).
2.3.2. Dng ch?y thng ? t?ng th?t:
+ Thng lin th?t (ventricular septal defect -VSD):
- Quanh mng.
- Vng ph?u.
- Bu?ng nh?n.
- Vng c? b.
+ Thng lin th?t km h? van ??ng m?ch ch?.
+ Thng lin th?t c lu?ng thng th?t tri-nh? ph?i.
2.3.3. Dng ch?y thng gi?a ??ng m?ch ch? v bn ph?i c?a tim:
- L? d ??ng m?ch vnh.
- V? ti phnh Valsalva.
- ??ng m?ch vnh tri b?t ngu?n t? thn ??ng m?ch ph?i.
2.3.4. Dng ch?y thng gi?a ??ng m?ch ch? v ??ng m?ch ph?i:
- C?a s? ph? ch? (l? d ph?-ch?: aortapulmonary window).
- Cn ?ng ??ng m?ch (t?n l?u ?ng ??ng m?ch: patent ductus arteriosus).
2.3.5. Dng ch?y thng trn m?t t?ng knh nh?-th?t:
2.4. B?nh tim b?m sinh c tm:
2.4.1. C t?ng tu?n hon ??ng m?ch ph?i:
- Hon v? ??i ??ng m?ch (transposition of great arteries).
- Th?t ph?i 2 ???ng ra ki?u taussig- bing.
- Thn chung ??ng m?ch (truncus arteriosus).
- N?i li?n b?t th??ng hon ton t?nh m?ch ph?i (t?nh m?ch ph?i v? l?c ch? ton ph?n: complete abnormal pulmonary venous connections).
Tm th?t ??n ??c (single ventricle) v?i s?c c?n m?ch ph?i th?p khng km h?p ??ng m?ch ph?i.
- Nh? chung.
- T? ch?ng Fallot ki?u khng l? van ??ng m?ch ph?i km t?ng tu?n hon bng h?.
- Khng l? van 3 l km thng lin th?t l? l?n (tricuspid atresia with large VSD).

2.4.2. Tu?n hon ??ng m?ch ph?i bnh th??ng hay gi?m:
+ Th?t tri tr?i:
- Khng l? van 3 l.
- Khng c l? van ??ng m?ch ph?i (pulmonary atresia) km vch lin th?t nguyn v?n.
- B?nh Ebstein.
- Tm th?t ??n ??c km h?p ??ng m?ch ph?i.
- N?i li?n b?t th??ng t?nh m?ch h? th?ng (abnormal systemic venous connections).
+ Th?t ph?i tr?i:
- Khng t?ng p ph?i.
- T? ch?ng Fallot.
- Tam ch?ng Fallot.
- Hon v? ??i ??ng m?ch c km h?p ??ng m?ch ph?i.
- Th?t ph?i 2 ???ng ra km h?p ??ng m?ch ph?i.
- Khng c van ??ng m?ch ph?i b?m sinh.
- Thng lin nh? v?i lu?ng thng ??o ng??c.
- Thng lin th?t v?i lu?ng thng ??o ng??c (ph?c h?p Eisenmenger).
- Cn ?ng ??ng m?ch ho?c c?a s? ph? ch? v?i dng ch?y thng ??o ng??c.
- Th?t ph?i 2 ???ng ra v?i s?c c?n m?ch ph?i cao.
- Hon v? ??i ??ng m?ch v?i s?c c?n m?ch ph?i cao.
- N?i li?n b?t th??ng ton ph?n t?nh m?ch ph?i v?i s?c c?n m?ch ph?i cao.
- Thi?u s?n tim tri (khng c l? van ??ng m?ch ch?, khng c l? van 2 l).
- L? d ??ng t?nh m?ch ph?i.
- T?nh m?ch ch? ?? vo nh? tri (n?i li?n b?t th??ng t?nh m?ch h? th?ng).
* Sau ?y l b?ng phn lo?i b?nh tim b?m sinh ? tu?i tr??ng thnh:
+ B?nh tim b?m sinh c t?n th??ng t?c ngh?n ??n gi?n:
- H?p l? van ??ng m?ch ch?.
- H?p l? van ??ng m?ch ph?i.
- H?p eo ??ng m?ch ch?.
- D? t?t ??ng m?ch vnh.
+ B?nh tim b?m sinh c lu?ng thng t? tri sang ph?i:
- Thng lin th?t.
- Thng lin nh?.
- Cn ?ng ??ng m?ch.
- Thng sn nh?-th?t.
+ B?nh tim b?m sinh c t?n th??ng ph?c t?p (th??ng c tm):
- B?nh Ebtein.
- T? ch?ng Fallot.
- Hon v? ??i ??ng m?ch.
- Tim m?t th?t.
3. Thng lin nh? (Atrial septal defect).
3.1. ??nh ngh?a:
Thng lin nh? l tnh tr?ng cn l? thng gi?a nh? tri v nh? ph?i ? vch lin nh?. ?y l b?nh tim b?m sinh hay g?p ? tu?i tr??ng thnh, ?i khi km theo cc d? t?t khc; n? b? nhi?u h?n nam.
3.2. Phn lo?i:
D?a vo v? tr l? thng, ng??i ta chia ra 3 lo?i:
- L? thng tin pht (primum atrial defect): l? thng n?m ? pha d??i l? b?u d?c, ngay b? trn c?a vng van nh?-th?t; hay g?p ? b?nh nhn c h?i ch?ng Down ho?c ? ng??i c b?nh h?p l? van 2 l b?m sinh t?o ra h?i ch?ng Lutembacher.
- L? thng th? pht (secundum atrial defect): l l? thng ? l? b?u d?c; l lo?i hay g?p trn lm sng.
- L? thng d?ng xoang t?nh m?ch (sinus venous defect): ? ph?n cao c?a vch lin nh?; th??ng ph?i h?p v?i d? t?t t?nh m?ch ph?i ?? v? nh? ph?i ho?c ?? vo t?nh m?ch ch? trn.
3.3. Huy?t ??ng h?c:
Bnh th??ng p l?c nh? tri (2-12 mmHg) cao h?n p l?c nh? ph?i (2-6 mmHg), nn khi cn l? thng lin nh? th dng mu s? ?i t? nh? tri sang nh? ph?i xu?ng th?t ph?i gy t?ng th? tch tm tr??ng th?t ph?i, t?ng l??ng mu ln ??ng m?ch ph?i s? gy t?ng p l?c ??ng m?ch ph?i. T?ng p l?c ??ng m?ch ph?i ko di s? d?n ??n t?ng s?c khng ton ph?i v p l?c nh? ph?i s? cao h?n nh? tri nn xu?t hi?n ??o shunt gy tri?u ch?ng tm trn lm sng.
3.4. Tri?u ch?ng lm sng:
- Tri?u ch?ng lm sng ph? thu?c vo kch th??c l? thng, m?c ?? t?ng gnh tm tr??ng th?t ph?i v m?c ?? t?ng p l?c ??ng m?ch ph?i.
- Giai ?o?n ??u, b?nh th??ng khng c bi?u hi?n lm sng nn khng ???c ch?n ?on, ??n t?n tu?i tr??ng thnh m?i pht hi?n ra b?nh. B?nh nhn th??ng pht tri?n bnh th??ng ho?c h?i nh? so v?i l?a tu?i.
- Khi c tri?u ch?ng c? n?ng: b?nh nhn th?y kh th?, hay ho ra mu, hay b? vim ph?i do t?ng p l?c ??ng m?ch ph?i.
- Nghe tim c ti?ng th?i tm thu ? van ??ng m?ch ph?i do c m?t l??ng mu ?i t? nh? tri qua nh? ph?i xu?ng th?t ph?i gy nn h?p ??ng m?ch ph?i c? n?ng. t khi c rung miu tm thu.
T?i ? van ??ng m?ch ph?i lun c T2 ?anh, tch ?i c? ??nh, khng thay ??i theo nh?p th? do t?ng p l?c ??ng m?ch ph?i.
- Cc tri?u ch?ng c?a suy tim ph?i: tim ph?i ??p ? m?i ?c [d?u hi?u Hartzer (+)], c th? c th?i tm thu ? m?i ?c do h? van 3 l c? n?ng [d?u hi?u Rivero-Carvalho (+)].
- Khi dng shunt ??i chi?u t? nh? ph?i sang nh? tri th th?y ti?ng th?i tm thu v ti?ng T2 gi?m c??ng ??, xu?t hi?n tm ? cc ??u ngn tay, chn, ? m?i, mi... Ngn tay di tr?ng l h?u qu? c?a s? thi?u xy ??ng m?ch ko di gy pht tri?n t? ch?c lin k?t v gin mao m?ch, t?nh m?ch ??u chi.
- ?i khi c lo?n nh?p tim cc lo?i.
- H?ng c?u v hematocrit t?ng d? gy hnh thnh c?c t?c ? t?nh m?ch. C?c t?c c th? di chuy?n v? tim ph?i gy t?c ??ng m?ch ph?i; c?c t?c c?ng c th? qua l? thng lin nh? gy t?c ??ng m?ch ngo?i vi; ho?c t?c ? ??ng m?ch no.
3.5. Tri?u ch?ng c?n lm sng:
3.5.1. X quang:
+ Chi?u tim th?y ??ng m?ch ph?i v?ng v ??p m?nh.
+ Ch?p tim-ph?i t? th? th?ng:
- Ph?i ? huy?t, km sng.
- ??ng m?ch ph?i v?ng.
- Gin cc cung nh? tri, nh? ph?i v th?t ph?i.
+ Ch?p X quang t? th? nghing tri:
- Nh? tri chn th?c qu?n (khi c u?ng baryt).
- Thu h?p hay m?t kho?ng sng sau x??ng ?c do th?t ph?i to.
3.5.2. ?i?n tim:
+ Lo?i th? pht: tr?c ph?i, t?ng gnh th?t ph?i, dy nh? ph?i, bl?c nhnh ph?i b His hon ton hay khng hon ton.
+ Lo?i l? thng tin pht: tim n?m ngang, QRS gin r?ng, tr?c tri, bl?c nh?-th?t c?p 1.
+ Lo?i xoang t?nh m?ch: nhi?u ngo?i tm thu nh?, bl?c nh?-th?t c?p 1.
C? 3 th? ??u c ph ??i th?t ph?i, gin nh? ph?i v nh? tri ? giai ?o?n sau.
3.5.3. Siu m tim:
Siu m tim c th? xc ??nh ???c t??ng ??i ch?c ch?n ch?n ?on thng lin nh?, nh?t l siu m qua th?c qu?n.
- Tri?u ch?ng tr?c ti?p: nhn ???c l? thng lin nh? v lu?ng mu ?i qua l? thng lin nh?, r nh?t l trn siu m qua th?c qu?n.
- Tri?u ch?ng gin ti?p:
. T?ng p l?c ??ng m?ch ph?i; ??o ng??c v?n ??ng vch lin th?t.
. Gin, ph ??i th?t ph?i.
B?ng siu m, ng??i ta cn c th? tnh ???c th? tch mu ?i qua l? thng trong m?i chu chuy?n tim, ?o ???c kch th??c l? thng gip cho ch? ??nh ph?u thu?t. Siu m c?n m c?ng r?t c gi tr? trong ch?n ?on.
3.5.4. Thng tim:
- C b??c nh?y v? ?? bo ho xy gi?a xoang t?nh m?ch v nh? ph?i. ? nh? ph?i, ?? bo ho
xy t?ng ln b?ng ho?c l?n h?n 10% so v?i xoang t?nh m?ch th c gi tr? ch?n ?on shunt tri sang ph?i.
- ?ng thng ?i ???c t? nh? ph?i sang nh? tri qua l? thng.
- B?m thu?c c?n quang ? ??ng m?ch ph?i (khi khng c h? ??ng m?ch ph?i) ho?c ? nh? tri th?y thu?c c?n quang sang ???c nh? ph?i.
3.6. Ch?n ?on.
3.6.1. Ch?n ?on xc ??nh:
Ch?n ?on xc ??nh d?a vo:
- Ti?ng th?i tm thu ? vng van ??ng m?ch ph?i.
- T2 ?anh, tch ?i c? ??nh khi th? su.
- ?i?n tim: tr?c ph?i, t?ng gnh th?t ph?i, bl?c nhnh ph?i b His.
- Siu m tim qua thnh ng?c ho?c qua th?c qu?n th?y l? thng, dng mu qua l? thng, t?ng p l?c ??ng m?ch ph?i.
- Thng tim v ch?p bu?ng tim, ?o ?? bo ho xy ? cc vng trong tim tm b??c nh?y v? ?? bo ho xy gi?a xoang t?nh m?ch v nh? ph?i, c gi tr? quy?t ??nh ch?n ?on.
3.6.2. Ch?n ?on phn bi?t:
- H?p l? van ??ng m?ch ph?i.
- Thng lin th?t.
- H? van 2 l.
3.7. Bi?n ch?ng, tin l??ng.
- B?i nhi?m ph?i-ph? qu?n, khi huy?t.
- Vim mng trong tim nhi?m khu?n (t g?p).
- Suy tim ph?i, t?c ??ng m?ch ph?i, t?c ??ng m?ch ngo?i vi, p xe no.
- Lo?i thng lin nh? xoang t?nh m?ch th th??ng lm cho b?nh nhn t? vong s?m nh?ng lo?i tin pht v th? pht th b?nh nhn s?ng g?n nh? bnh th??ng.
3.8. D? phng v ?i?u tr?.
3.8.1. D? phng:
Trnh cc tc nhn gy b?nh tim b?m sinh ? nh?ng thng ??u c?a thai.
3.8.2. ?i?u tr?:

Adult Congenital Heart Disease Cardiac Cath Interventions Adult Congenital Heart Disease Operations
+ N?i khoa:
- Ch?ng nhi?m khu?n h h?p, lm ch?m th?i gian gy t?ng p l?c ??ng m?ch ph?i, d? phng vim mng trong tim nhi?m khu?n.
- Phng ch?ng lo?n nh?p tim.
- ?i?u tr? suy tim.
- D? phng t?c m?ch b?ng thu?c ?c ch? k?t dnh ti?u c?u: aspirin 0,1-0,5g/ngy.
+ Ngo?i khoa:
- ?ng l? thng b?ng v?t li?u nhn t?o. Th??ng m? khi b?nh nhn ln 3-6 tu?i, khi ch? s? dng mu ph?i/dng mu ??ng m?ch ch? > 2,0, ch?a c ??o shunt.
- Khng m? n?u b?nh nhn c l? thng nh? v c d? t?t n?ng ? ??ng m?ch ph?i.
- N?u c h?p l? van 2 l th s?a ho?c thay van.
- C th? dng thng tim ?? b?t l? thng b?ng d.
4. Thng lin th?t (Ventricular septum defect):
4.1. ??nh ngh?a:
Thng lin th?t l t?n t?i m?t l? thng ? vch lin th?t.
B?nh hay g?p, chi?m kho?ng 18% t?ng s? b?nh tim b?m sinh.
4.2. Phn lo?i:
Friedman W.P chia ra 5 th? thng lin th?t:
- Th? 1: l? thng n?m ? pha trn v tr??c c?u b? c? (Crista), ngay d??i van ??ng m?ch ph?i v l van ??ng m?ch vnh tri c?a ??ng m?ch ch?.
- Th? 2: l? thng n?m ? pha sau v trn c?a c?u b? c?, g?i l thng lin th?t ph?n mng, lin quan ch?t ch? v?i ??ng m?ch ch?.
- Th? 3: l? thng ? pha sau van 3 l, lin quan v?i l sau van 2 l.
- Th? 4: l? thng ? ph?n c? c?a vch lin th?t, d??i cc tr? c? c?a van 3 l.
- Th? 5: c nhi?u l? thng nh? ? ph?n c? g?n m?m tim c?a vch lin th?t.
4.3. Huy?t ??ng h?c:
?? l?n c?a l? thng v m?c ?? t?ng p l?c ??ng m?ch ph?i quy?t ??nh tri?u ch?ng lm sng. Mu t? th?t tri (v?i p l?c tm thu 100-140 mmHg) sang th?t ph?i (c p l?c tm thu 15-30 mmHg) qua l? thng gy dng shunt tri sang ph?i. S? t?ng th? tch th?t tri v th?t ph?i ph? thu?c vo s?c khng c?a ph?i. V? lu di, s?c khng c?a ph?i t?ng lm cho p l?c th?t ph?i cao h?n th?t tri, lc ? dng shunt ??o ng??c t? th?t ph?i sang th?t tri, lc ny xu?t hi?n tm trn lm sng v g?i l ph?n ?ng Eisenmenger.
Khi l? thng qu l?n, hai bu?ng th?t coi nh? l m?t, lm p l?c ??ng m?ch ph?i t?ng s?m, ??o shunt s?m, b?nh nhn t? vong khi cn nh?.
N?u kch th??c l? thng v?a, ??o shunt ? tu?i thanh nin, b?nh nhn th??ng km pht tri?n th? l?c do thi?u xy.
N?u l? thng nh?, b?nh nhn s?ng lu v ch?m ??o shunt, g?i l b?nh Roger.
4.4. Tri?u ch?ng lm sng:
- Tri?u ch?ng lm sng bi?u hi?n s?m hay mu?n ph? thu?c vo kch th??c l? thng v m?c ?? t?ng p l?c ??ng m?ch ph?i.
- L? thng b th giai ?o?n khng tri?u ch?ng ko di, v? sau m?i c tri?u ch?ng kh th?, m?t m?i khi g?ng s?c.
- L? thng v?a v l?n th hay gy nhi?m khu?n h h?p, ch?m l?n; tnh tr?ng tm xu?t hi?n s?m (giai ?o?n ??u ch? tm khi g?ng s?c, v? sau tm th??ng xuyn), ngn tay di tr?ng, hay c t?c ??ng m?ch ph?i, ho ra mu, ?au ng?c.
- L?ng ng?c th??ng bi?n d?ng r do tim to t? khi b?nh nhn cn b.
- Nghe th?y th?i tm thu m?nh ? lin s??n III-IV c?nh ?c tri lan theo hnh nan hoa, th??ng c rung miu tm thu. T?i vng van ??ng m?ch ph?i c T2 ?anh v tch ?i do t?ng p l?c ??ng m?ch ph?i; c th? c ti?ng th?i tm thu nh? do h?p l? van ??ng m?ch ph?i c? n?ng.
? m?m tim c th? c ti?ng T3, rng tm tr??ng nh? do t?ng kh?i l??ng mu qua van 2 l t?o nn h?p l? van 2 l c? n?ng.
4.5. Tri?u ch?ng c?n lm sng:
4.5.1. X quang tim-ph?i:
- Khi l? thng nh? th X quang tim-ph?i bnh th??ng.
- Khi l? thng v?a ho?c l?n:
. Nh? tri gin.
. 2 th?t gin.
. ??ng m?ch ph?i gin; t?ng p l?c ??ng m?ch ph?i, cn ??ng m?ch ch? v?n bnh th??ng ho?c h?i x?p.
4.5.2. ?i?n tim:
- T?ng gnh 2 th?t.
- Gin nh? tri.
4.5.3. Siu m tim:
- Hnh ?nh tr?c ti?p: th?y l? thng ? vch lin th?t, th?y lu?ng mu ?i qua l? thng.
- Hnh ?nh gin ti?p: r?i lo?n v?n ??ng vch lin th?t, gin nh? tri v 2 th?t.
- Hay c h? van ??ng m?ch ch? k?t h?p ? th? 1.
- Siu m cn cho php xc ??nh kch th??c l? thng, dng mu qua l? thng trong m?t nht bp, ?o ???c p l?c ??ng m?ch ph?i ?? gip ch? ??nh ?i?u tr?.
4.5.4. Thng tim:
?? ch?n ?on xc ??nh: ?o ???c p l?c ??ng m?ch ph?i; xc ??nh v? tr, kch th??c l? thng.
- C b??c nh?y ?? bo ho xy gi?a nh? ph?i v th?t ph?i.
- ?ng thng c th? ??a t? th?t ph?i sang th?t tri v ng??c l?i.
- Ch?p bu?ng th?t th?y mu ?i qua l? thng.
4.6. Ch?n ?on xc ??nh:
4.6.1. Ch?n ?on:
- Ti?ng th?i tm thu m?nh ? lin s??n III-IV c?nh ?c tri lan theo hnh nan hoa, th??ng c rung miu tm thu.
- X quang, ?i?n tim: gin nh? tri v dy 2 th?t.
- Siu m: nhn th?y l? thng v dng mu qua l? thng ? vch lin th?t.
- Thng tim v ch?p c?n quang bu?ng tim.
4.6.2. Ch?n ?on phn bi?t:
- H? van 2 l, h? van 3 l.
- H?p l? van ??ng m?ch ch? v h?p l? van ??ng m?ch ph?i.
- Thng lin nh?.
4.7. Bi?n ch?ng, tin l??ng:
- Hay b? nhi?m khu?n h h?p.
- T? l? b? vim mng trong tim nhi?m khu?n r?t cao.
- Suy tim.
- T?c m?ch, p xe no.
- C kho?ng 40-45% thng lin th?t t? ?ng l?i khi ??n tu?i tr??ng thnh.
4.8. D? phng v ?i?u tr?.
4.8.1. D? phng:
Gi?ng nh? thng lin nh?.
4.8.2. ?i?u tr?:
+ N?i khoa:
- Phng ch?ng nhi?m khu?n h h?p v vim mng trong tim nhi?m khu?n.
- ?i?u tr? suy tim.
- Phng t?c m?ch.
+ Ngo?i khoa:
- ?ng l? thng b?ng v?t li?u nhn t?o ???c ch? ??nh khi ch? s? dng mu ph?i/dng mu ngo?i vi ? 2, b?t ??u c t?ng p l?c ??ng m?ch ph?i, tu?i c?a b?nh nhn ???c ph?u thu?t t?t nh?t l t? 2-10.
- ?ng l? thng lin th?t b?ng thng tim ??t d t?i l? thng.
5. T?n t?i ?ng ??ng m?ch (Patent ductus arteriosus).
5.1. ??nh ngh?a:
Tr? s? sinh sau ?? 3 thng m v?n t?n t?i ?ng thng gi?a ??ng m?ch ch? v ??ng m?ch ph?i th ???c g?i l cn t?n t?i ?ng ??ng m?ch (?ng Botal).
B?nh chi?m 13% trong cc b?nh tim b?m sinh; n? b? nhi?u h?n nam.
5.2. Phn lo?i:
?ng ??ng m?ch ???c hnh thnh trong bo thai cng v?i ??ng m?ch ph?i v cc nhnh ??u tin vng g?c ??ng m?ch ch?. C?n c? vo v? tr ?? vo ??ng m?ch ph?i, ng??i ta chia ra:
- Tp 1: t?n t?i ?ng ??ng m?ch ?? vo ??ng m?ch ph?i tri.
- Tp 2: t?n t?i ?ng ??ng m?ch ?? vo ng ba ch? ??ng m?ch ph?i g?c chia ra ??ng m?ch ph?i ph?i v tri.
- Tp 3: t?n t?i ?ng ??ng m?ch ?? v? ??ng m?ch ph?i g?c.
t khi ?ng ??ng m?ch ?? v? ??ng m?ch ph?i ph?i.
5.3. Sinh b?nh h?c, huy?t ??ng h?c:
- V?n ?? ?ng ?ng ??ng m?ch sau sinh: thnh c?a ?ng ??ng m?ch ???c c?u trc b?i t? bo c? tr?n, cc t? bo ny m?t kh? n?ng co gin n?u c kch thch c?a adrenalin, noradrenalin, n?ng ?? v phn p xy cao, kinin, bradykinin v c? cc kch thch c? h?c, ?i?n h?c. Bnh th??ng, ?ng ??ng m?ch ?ng kn 8-12 gi? sau ??. Ng??i ta cn th?y, khi ph?i b?t ??u h h?p lm phn p xy t?ng ??t ng?t, ph?i gi?i phng ra cc ch?t c ho?t tnh trn m?ch mu (nh?t l prostaglandin v prostacycline) ?c ch? pht tri?n n?i m?c c?a ?ng thng lm ko di th?i gian bt t?c ?ng thng gy ra b?nh t?n t?i ?ng ??ng m?ch.
- ?a s? l t?n t?i ?ng ??ng m?ch ??n thu?n, ?i khi k?t h?p v?i cc d? t?t b?m sinh khc nh?: h?p eo ??ng m?ch ch?, thng lin th?t, h?p ??ng m?ch ph?i, h?p ??ng m?ch ch?...
- Dng mu ?i qua ?ng ??ng m?ch ph? thu?c vo: chnh p gi?a ??ng m?ch ch? v ??ng m?ch ph?i, ???ng knh v ?? di ?ng ??ng m?ch.
Bnh th??ng p l?c ??ng m?ch ch? th tm thu l 100-140 mmHg, th tm tr??ng l 60-70 mmHg; cn p l?c ??ng m?ch ph?i th tm thu l 15-30 mmHg, th tm tr??ng l 4-15 mmHg nn dng mu s? ?i t? ??ng m?ch ch? sang ??ng m?ch ph?i c? 2 th (tm thu v tm tr??ng) t?o dng shunt tri sang ph?i. Sau ny, khi p l?c ??ng m?ch ph?i t?ng cao h?n ??ng m?ch ch? s? c ??o shunt v gy tm trn lm sng.
5.4. Tri?u ch?ng lm sng:
- Giai ?o?n ??u: tri?u ch?ng c? n?ng ch?a xu?t hi?n n?u ?ng ??ng m?ch nh?; cn n?u ?ng ??ng m?ch l?n th s? s?m c tri?u ch?ng kh th?, t?c ng?c, ho ra mu, vim ph?i...
- ? lin s??n II-III c?nh ?c tri c ti?ng th?i lin t?c, c??ng ?? m?nh ln ? th tm thu. Ti?ng th?i ny lan ra xung quanh. T?i ?y th??ng s? th?y rung miu. ?i khi nghe th?y ti?ng rung tm tr??ng ? m?m tim do mu qua ?ng ??ng m?ch v? ph?i r?i ?? vo nh? tri lm cho kh?i l??ng mu t?ng ln gy h?p l? van hai l c? n?ng. C th? nghe ???c ti?ng th?i tm thu ? vng van ??ng m?ch ch? do h?p l? van ??ng m?ch ch? c? n?ng.
- C th? c cc tri?u ch?ng ??ng m?ch ngo?i vi gi?ng nh? h? l? van ??ng m?ch ch?: m?ch Corrigan; huy?t p tm thu t?ng, huy?t p tm tr??ng gi?m; d?u hi?u l?p lo mng tay...
- p l?c ??ng m?ch ph?i t?ng d?n s? gy ??o shunt ph?i sang tri, lc ny trn lm sng s? xu?t hi?n tm, ngn tay di tr?ng, t?ng h?ng c?u v hematocrit.
5.5. Tri?u ch?ng c?n lm sng:
5.5.1. X quang:
- Chi?u tim-ph?i th?y cung ??ng m?ch ph?i ??p m?nh.
- Ch?p tim-ph?i: cung nh? tri v th?t tri gin to, cung ??ng m?ch ch? gin r?ng, hnh ?nh t?ng p l?c ??ng m?ch ph?i, ph?i km sng.
5.5.2. ?i?n tim:
Ph ??i th?t tri, gin nh? tri v nh? ph?i.
5.5.3. Tm thanh ??:
C ti?ng th?i lin t?c, ??nh cao nh?t ? th tm thu.
5.5.4. Siu m tim:
- Hnh ?nh gin ti?p: ???ng knh nh? tri v th?t tri t?ng.
- C th? th?y ???c ?ng ??ng m?ch trn siu m 2D, ?o ???c ???ng knh v ?? di ?ng ??ng m?ch.
- Siu m Doppler xc ??nh ???c lu?ng mu ?i qua ?ng ??ng m?ch t? ??ng m?ch ch? ??n ??ng m?ch ph?i; c th? ?o ???c th? tch mu qua ?ng thng ??ng m?ch b?ng siu m Doppler.
5.5.5. Thng tim:
- C th? ??a ???c ?ng thng t? ??ng m?ch ch? sang ??ng m?ch ph?i.
- Ch?p c?n quang ??ng m?ch ch? th?y thu?c sang ???c ??ng m?ch ph?i.
- Phn p xy ? ??ng m?ch ph?i t?ng.
5.6. Ch?n ?on:
5.6.1. Ch?n ?on xc ??nh:
- Lm sng: nghe th?y ti?ng th?i lin t?c, m?nh ln ? th tm thu ? lin s??n II-III c?nh ?c tri; s? c rung miu. C th? c tm, ngn tay di tr?ng n?u c ??o shunt.
- X quang: gin nh? tri v th?t tri, t?ng p l?c ??ng m?ch ph?i.
- ?i?n tim: gin nh? tri, dy th?t tri.
- Siu m: tm ???c ?ng ??ng m?ch, th?y dng mu qua ?ng ??ng m?ch trn siu m Doppler.
- Thng tim gip ch?n ?on ch?c ch?n.
Ch?n ?on kh khi c t?ng p l?c ??ng m?ch ph?i m p l?c ny cn b?ng gi?a ??ng m?ch ph?i v ??ng m?ch ch? gy m?t ti?ng th?i lin t?c.
5.6.2. Ch?n ?on phn bi?t:
- Thng lin th?t.
- Thng lin nh?.
- H? van ??ng m?ch ch?, h? v h?p l? van ??ng m?ch ch?.
- H? v h?p l? van ??ng m?ch ph?i.
5.7. Bi?n ch?ng v tin l??ng:
- R?t hay g?p vim mng trong tim nhi?m khu?n, m giai ?o?n ??u l vim n?i m?c ??ng m?ch nhi?m khu?n.
- Vim ph?i ti di?n.
- Gin phnh ??ng m?ch ph?i.
- Vi ho, ??t ho?c v? ?ng ??ng m?ch.
- Suy tim.
- T?c ??ng m?ch ph?i ho?c t?c ??ng m?ch ngo?i vi khi ??o shunt.
5.8. ?i?u tr?:
5.8.1. ?i?u tr? n?i khoa:
- Nh?ng tu?n ??u sau ?? n?u pht hi?n cn t?n t?i ?ng ??ng m?ch th dng indomethacin ho?c ibuprofen ?? ?c ch? prostaglandin v prostacycline s? gy tc d?ng co th?t t?o ?i?u ki?n ?ng ???c ?ng thng ??ng m?ch.
- Phng ch?ng vim mng trong tim nhi?m khu?n.
- Phng ch?ng vim ph?i, t?c m?ch.
5.8.2. ?i?u tr? ngo?i khoa:
Th?t, bu?c, c?t ?ng ??ng m?ch. Nn m? s?m tr??c khi c t?ng p l?c ??ng m?ch ph?i gy ??o shunt.
5.8.3. ?i?u tr? b?ng thng tim:
?t nt l? thng l th? thu?t an ton, hi?u qu?, ti?n hnh ? tr? d??i 2 tu?i s? r?t t?t.
6. H?p ??ng m?ch ch? b?m sinh (Congenital aortic stenosis).
6.1. ??i c??ng:
+ C th? c h?p ??ng m?ch ch? tr??c van, t?i van v trn van b?m sinh. ?i khi c ph?i h?p v?i cc t?t b?m sinh khc.
+ Ng??i ta chia h?p ??ng m?ch ch? lm 3 tp:
- Tp 1: h?p t?i van ??ng m?ch ch?.
. Van ??ng m?ch ch? ch? c m?t l van.
. Van ??ng m?ch ch? ch? c 2 l van.
. Van ??ng m?ch ch? c 3 l van.
- Tp 2: h?p d??i van.
. H?p mng d??i van.
. Ph ??i vch.
- Tp 3: h?p trn van.
. H?p mng trn van.
. Th?t h?p trn van.
. Thi?u s?n ??ng m?ch ch? trn van.
H?p ??ng m?ch ch? b?m sinh chi?m kho?ng 5% cc b?nh tim b?m sinh; tr? em nam gi?i g?p nhi?u h?n n? gi?i. Tp 2 v 3 hay c tnh gia ?nh. Tp 1 lo?i c hai l van th??ng km theo h? van ??ng m?ch ch?. Tp 3 hay ph?i h?p v?i d? t?t van 2 l.
6.2. B?nh sinh:
H?p l? van ??ng m?ch ch? gy t?ng p l?c tm thu th?t tri, gi?m dng mu ?i qua ch? h?p gy ph ??i th?t tri, gi?m p l?c ??ng m?ch, gi?m tr??ng l?c ??ng m?ch ngo?i vi; nn b?nh nhn hay b? ng?t.
Sau d?n nh? tri c?ng gin, tim ??p m?nh ? m?m nn c th? t?o ra ti?ng T4.
6.3. Tri?u ch?ng:
6.3.1. Tri?u ch?ng lm sng:
- Tri?u ch?ng c? n?ng ph? thu?c vo m?c ?? h?p: m?t m?i, ?au ng?c, ng?t, kh th?.
- Tri?u ch?ng th?c th?: Huy?t p th?p v m?ch ngo?i vi y?u. ??ng m?ch c?nh ??p y?u, s? ? lin s??n II ph?i v lin s??n III tri c?nh x??ng ?c c rung miu tm thu. C th?i tm thu m?nh lan ln ??ng m?ch ch? v d?c b? tri c?nh ?c, ti?ng T2 m?.
Khi h?p d??i van s? nghe ???c ti?ng th?i tm thu ? lin s??n IV-V c?nh ?c tri. Khi ph ??i vch lin th?t s? lm h?p ph?n t?ng mu c?a th?t ph?i gy ra ti?ng th?i tm thu ? vng van ??ng m?ch ph?i. T?i m?m tim c ti?ng T3 v ti?ng th?i tm thu do h? van 2 l c? n?ng (do nh? tri v th?t tri gin).

6.3.2. Tri?u ch?ng c?n lm sng:
- X quang: gin th?t tri v nh? tri, cung ??ng m?ch ch? x?p; v? sau s? gin c? th?t ph?i.
- ?i?n tim: gin nh? tri, dy th?t tri.
Ring tp 2 c ph ??i vch lin th?t nn sng Q su ? DII, DIII, V5, V6 (c th? nh?m v?i nh?i mu c? tim c?), ?i khi c h?i ch?ng Wolf-Parkinson-White.
- Siu m tim: th?y tim tri ph ??i v gin, nh? tri gin. Siu m c?ng ch?n ?on ???c t?ng tp c?a b?nh:
. Tp 1: van ??ng m?ch ch? m? khng st thnh, bin ?? m? van gi?m; trn siu m 2D xc ??nh ???c van ??ng m?ch ch? c 1, 2 ho?c 3 l van.
. Tp 2: th?y ???c m?t mng ng?n d??i van hnh c?a g ho?c ph ??i vch lin th?t ph?n t?ng mu.
. Tp 3: th?y mng ng?n trn van ho?c th?y kch th??c c?a ??ng m?ch ch? trn van nh? l?i, thnh th?t dy.
- Thng tim: ?o th?y chnh p l?c tm thu gi?a bu?ng th?t v ??ng m?ch ch? t?ng (nh?ng th??ng < 30 mmHg).
6.4. Ch?n ?on:
6.4.1. Ch?n ?on xc ??nh:
- Lm sng: ti?ng th?i tm thu m?nh ? vng van ??ng m?ch ch?, th??ng c rung miu tm thu; T2 m? ? vng van ??ng m?ch ch?. Huy?t p ngo?i vi th?p, huy?t p tay ph?i cao h?n tay tri.
- X quang: th?t tri v nh? tri gin to.
- ?i?n tim: gin nh? tri, dy th?t tri.
- Siu m tim: ch?n ?on kh chnh xc.
- Thng tim: chnh l?ch p l?c gi?a th?t tri v ??ng m?ch ch? t?ng d??i 30 mmHg.
6.4.2. Ch?n ?on phn bi?t:
- Thng lin th?t.
- H? van 3 l.
- H?p, phnh gin ??ng m?ch c?nh.
6.5. ?i?u tr?:
6.5.1. ?i?u tr? n?i khoa:
Giai ?o?n ??u ?i?u tr? b?ng n?i khoa t hi?u qu?, ch? phng ch?ng vim mng trong tim nhi?m khu?n.
Khi ? c ?au ng?c, ng?t th dng thu?c ?c ch? bta ?? lm gi?m s? h?p t?c: dng propranolol 40mg x 1 vin/ngy.
6.5.2. ?i?u tr? ngo?i khoa:
Ph?u thu?t s?a d? t?t, c?t ph?n h?p hay thay van ??ng m?ch ch?. T? l? t? vong trong ph?u thu?t cn cao t?i 5-10%.
7. H?p ??ng m?ch ph?i (Congenital pulmonary stenosis).
7.1. ??i c??ng:
T? l? h?p ??ng m?ch ph?i g?p 10-15% trong cc th? b?nh tim b?m sinh. L hi?n t??ng h?p ???ng ra c?a th?t ph?i. C 4 th? b?nh:
- H?p l? van ??ng m?ch ph?i: hay g?p nh?t.
- H?p d??i ph?n ph?u ??ng m?ch ph?i.
- H?p ph?n ph?u ??ng m?ch ph?i.
- H?p trn van.
B?nh ??c l?p ho?c km theo d? t?t van v thng lin th?t.
Th? h?p d??i ph?n ph?u ??ng m?ch ph?i gi?ng nh? chia th?t ph?i ra thnh 2 bu?ng, p l?c ? hai bu?ng khc nhau; th??ng ph?i h?p v?i thng lin th?t.
Th? h?p trn van ??ng m?ch ph?i th??ng km theo h?p nhnh ??ng m?ch ph?i ho?c t? ch?ng Fallot v t?n t?i ?ng ??ng m?ch.
7.2. B?nh sinh:
? giai ?o?n ??u c?a thai nhi, c th? ng??i m? b? nhi?m virut Rubeol ho?c r?i lo?n chuy?n ho canxi v vitamin D lm gi?m kh? n?ng pht tri?n bnh th??ng c?a ??ng m?ch ph?i.
H?p ??ng m?ch ph?i lm t?ng s?c khng, d?n ??n t?ng p l?c tm thu th?t ph?i. N?u h?p n?ng th lm t?ng th?i gian t?ng mu th?t ph?i gy ph ??i th?t ph?i v nh? ph?i, bin ?? sng a c?a t?nh m?ch t?ng ln. Do t?ng p l?c nh? ph?i c th? gy ra m? l? thng lin nh? ? h? b?u d?c, t?o ra shunt ph?i sang tri gy tm ? lm sng v suy tim ? tr? c h? van 3 l c? n?ng do th?t ph?i gin to.
7.3. Lm sng v c?n lm sng:
7.3.1. Lm sng:
Khi h?p nh? th tri?u ch?ng lm sng khng bi?u hi?n r; n?u h?p v?a v n?ng th tri?u ch?ng lm sng s? ?i?n hnh.
- Th? h?p l? van ??ng m?ch ph?i: c ti?ng th?i tm thu t??ng ??i m?nh ? lin s??n II-III c?nh ?c tri hay hm trn x??ng ?c, ti?ng th?i c tnh ch?t th rp; th??ng c rung miu tm thu ? vng van ??ng m?ch ph?i; ti?ng T2 bnh th??ng ho?c m?.
T?i m?i ?c: tim ph?i ??p m?nh, T1 v?n bnh th??ng.
Xu?t hi?n tm khi g?ng s?c, sau ? l c? khi ngh? ng?i v c ngn tay di tr?ng.
Giai ?o?n c suy tim ph?i: xu?t hi?n ph, gan to, t?nh m?ch c? n?i; c ti?ng th?i tm thu ? m?i ?c do h? van 3 l c? n?ng.
- Th? h?p ph?u hay d??i ph?u ??ng m?ch ph?i: c ti?ng th?i tm thu t?ng mu m?nh h?n v ? vng th?p h?n so v?i th? h?p l? van ??ng m?ch ph?i.
- Th? h?p trn van ??ng m?ch ph?i: ti?ng th?i tm thu nghe ? v? tr xa vng tr??ng ph?i, r h?n ? vng van ??ng m?ch ph?i; c khi nghe th?y ti?ng th?i lin t?c v mu ?i qua nhnh ??ng m?ch ph?i b? h?p; T2 bnh th??ng v p l?c ??ng m?ch ph?i th?p.
7.3.2. C?n lm sng:
- X quang: n?u h?p nh? th ch? th?y gin nh? ??ng m?ch ph?i sau ch? h?p. N?u h?p v?a v n?ng th th?y th?t ph?i v nh? ph?i gin to, ph?i sng, vi ho van ??ng m?ch ph?i.
- ?i?n tim: giai ?o?n ??u c?a h?p ??ng m?ch ph?i m?c ?? nh? th p l?c th?t ph?i khng cao nn ?i?n tim bnh th??ng.
Sau ?, khi p l?c th?t ph?i t?ng cao s? c ph ??i th?t ph?i. Khi p l?c th?t ph?i > 120 mmHg th sng T ??o ng??c v?i R ? cc ??o trnh tr??c tim.
- Thng tim: khi thng tim ph?i th?y h?p ??ng m?ch ph?i, c b??c gi?m p l?c gi?a th?t ph?i v ??ng m?ch ph?i.
- Siu m: c th? nhn th?y v? tr h?p v ? ? dng mu t? th?t ph?i ?i vo ??ng m?ch ph?i t?ng ln v c hnh ?nh r?i dng (th?y c mu kh?m do t?c ?? dng mu t?ng).
7.4. Ch?n ?on:
7.4.1. Ch?n ?on xc ??nh:
- Lm sng: lin s??n II-III c?nh ?c tri c ti?ng th?i tm thu m?nh, T2 m?, c rung miu tm thu.
- X quang: nh? ph?i v th?t ph?i to, ph?i sng.
- ?i?n tim: dy th?t ph?i, gin nh? ph?i.
- Siu m: th?y hnh ?nh h?p ??ng m?ch ph?i, dng mu qua ch? h?p ??ng m?ch ph?i c mu kh?m trn siu m Doppler.
- Thng tim: p l?c th?t ph?i t?ng, p l?c ??ng m?ch ph?i gi?m. Ch?p bu?ng tim c?n quang th?y ?o?n h?p.
7.4.2. Ch?n ?on phn bi?t:
- Thng lin nh?: trn lm sng khng c tm, ti?ng th?i tm thu ? th?p, ph?i khng sng m m?. Thng tim v siu m gip ch?n ?on ch?c ch?n.
- T? ch?ng Fallot.
- Thng lin th?t: p l?c ??ng m?ch ph?i t?ng nhi?u, siu m v thng tim gip ch?n ?on ch?c ch?n.
- H?p ??ng m?ch ch? b?m sinh.
7.5. ?i?u tr?:
7.5.1. ?i?u tr? n?i khoa:
?i?u tr? b?ng n?i khoa b?nh h?p ??ng m?ch ph?i t c hi?u qu?; ch? khi b?nh nhn c tm v suy tim ph?i th ph?i ?i?u tr? suy tim v dng thu?c ch?ng ?ng.
7.5.2. ?i?u tr? ngo?i khoa:
S?a cc d? t?t khi c ?? chnh p l?c gi?a th?t ph?i v ??ng m?ch ph?i l?n h?n 50 mmHg.
7.5.3. Nong ??ng m?ch ph?i b?ng bng qua thng tim: Th??ng lm ? tr? nh?.
8. H?p eo ??ng m?ch ch? (Coarctation of the aorta).
8.1. ??i c??ng v phn lo?i:
H?p eo ??ng m?ch ch? g?p t? 5-10% trong cc b?nh tim b?m sinh.
Th??ng l h?p ? ph?n xu?ng hay ?o?n gi?a ??ng m?ch ch? ng?c, ?i khi c h?p ??ng m?ch ch? b?ng pha trn ??ng m?ch thn km theo h?p ??ng m?ch thn.
T?i ch? h?p, ??ng m?ch ch? th?t l?i ho?c thi?u s?n ??ng m?ch ch? m?t ?o?n di. C th? c m?t hay nhi?u ch? h?p ? m?t b?nh nhn; c th? km theo cc d? t?t khc nh? van ??ng m?ch ch? 2 l, cn ?ng ??ng m?ch...

D?a vo v? tr ?ng thng ??ng m?ch lm m?c, ng??i ta chia h?p eo ??ng m?ch ch? thnh 2 tp.
- Tp 1: ? tr? em, hay g?p h?p eo ??ng m?ch ch? tr??c ?ng ??ng m?ch.
- Tp 2: ? ng??i l?n, h?p ??ng m?ch ch? sau ?ng ??ng m?ch.
8.2. B?nh sinh:
Hnh thnh v? tr h?p eo ??ng m?ch ch? t??ng ?ng v?i th?i gian chia nhnh th? t? c?a ??ng m?ch ch? trong th?i k? bo thai; th??ng do b?t th??ng c?a nhi?m s?c th?. V v?y, h?p eo ??ng m?ch ch? hay g?p cng v?i h?i ch?ng Turner; nam b? nhi?u h?n n? 4-5 l?n.

Ph?n tr??c ch? h?p c t?ng huy?t p do l do c? h?c v do vai tr c?a thn b? thi?u mu gy ra.
Tu?n hon bn phong ph n?i trn v d??i ch? h?p, nh?t l h? ??ng m?ch lin s??n v ??ng m?ch v trong. T?ng huy?t p tm thu ? h? ??ng m?ch l?ng ng?c gy ph ??i th?t tri; v? sau gy gin th?t tri v suy tim ? tr?; c th? dy, gin v v? cc d? t?t m?ch mu trong s?.
Sau ch? h?p, ??ng m?ch gin ra, c th? c phnh, bc tch v hay b? rch v? ??ng m?ch ch?. Ph?n ln c?a quai ??ng m?ch ch? c?ng b? gin gy h? van ??ng m?ch ch?, nh?t l khi c ph?i h?p v?i t?t van ??ng m?ch ch? ch? c hai l van.
8.3. Tri?u ch?ng lm sng v c?n lm sng:
8.3.1. Tri?u ch?ng lm sng:
B?nh th??ng g?p ? nam gi?i; th? l?c ng??i b?nh v?n pht tri?n bnh th??ng. ?o p l?c ??ng m?ch trn ch? h?p t?ng cao. B?nh nhn b? ?au ??u nhi?u, ch?y mu cam, m?t m?i, huy?t p chi trn t?ng cao trong khi hai chn lun c c?m gic l?nh; ??ng m?ch ?i ??p y?u ho?c s? khng th?y; huy?t p chi d??i th?p h?n huy?t p ? cnh tay ph?i; huy?t p ??ng m?ch cnh tay tri c?ng th?p h?n bn ph?i; ??ng m?ch c?nh tri ??p y?u h?n ??ng m?ch c?nh ph?i; c ti?ng th?i tm thu v rung miu tm thu ? pha sau thnh ng?c do cung l??ng mu c?a ??ng m?ch lin s??n v cc ??ng m?ch c vai tr tu?n hon bn, th??ng nghe ???c ti?ng th?i r nh?t ? d??i x??ng b? vai. Khi h?p eo ??ng m?ch ch? nh? th ti?ng th?i tm thu nh?. N?u h?p v?a v n?ng th ti?ng th?i tm thu s? m?nh, nghe r ? c?nh s?ng l?ng bn tri, lan d?c theo ??ng m?ch ch?; T2 ?anh.
8.3.2. Tri?u ch?ng c?n lm sng:
- X quang: Trn phim th?ng th?y cung sau x??ng s??n I, II, III ? b? d??i (n?i c ??ng m?ch lin s??n) b? gin r?ng, c th? c hnh r?ng c?a (ch? r khi b?nh nhn ? 10-12 tu?i). Ph?n ln c?a ??ng m?ch ch? gin r?ng.
Trn phim nghing th?y ch? h?p c?a ??ng m?ch ch? th?t l?i, sau ch? h?p ??ng m?ch ch? phnh gin ra.
Ch?p c?n quang ??ng m?ch ch? b?ng ?ng catheter xc ??nh ???c v? tr, m?c ?? h?p, chnh p qua ch? h?p; tu?n hon bn v pht hi?n xem c t?n t?i ?ng ??ng m?ch khng.
- ?i?n tim: t?ng gnh th?t tri xu?t hi?n s?m.
8.4. Bi?n ch?ng, tin l??ng:
- Suy tim tri.
- ??t v?, phnh ??ng m?ch ch?.
- Vim mng trong tim, vim n?i m?c ??ng m?ch ch? nhi?m khu?n.
- Xu?t huy?t no.
8.5. Ch?n ?on:
8.5.1. Ch?n ?on xc ??nh:
- Lm sng: t?ng huy?t p chi trn (t?ng r?t cao), huy?t p chi d??i gi?m. C ti?ng th?i tm thu t?ng mu ? sau l?ng lan d?c ??ng m?ch ch? t??ng ?ng v?i ch? h?p.
- X quang: gin g?c ??ng m?ch ch?, h?p eo ??ng m?ch ch?, gin ??ng m?ch sau ch? h?p. Hnh ?nh g?m nh?m cung sau x??ng s??n do ??ng m?ch lin s??n t?ng p l?c v gin, xem r khi ch?p c?n quang.
- ?i?n tim: t?ng gnh th?t tri.
8.5.2. Ch?n ?on phn bi?t:
- T?ng huy?t p.
- Thng lin th?t.
- Cn ?ng ??ng m?ch.
8.6. ?i?u tr?:
8.6.1. ?i?u tr? n?i khoa:
Ch? ?i?u tr? n?i khoa khi c bi?n ch?ng: vim n?i m?c ??ng m?ch, vim mng trong tim nhi?m khu?n, suy tim, h? huy?t p...
8.6.2. ?i?u tr? ngo?i khoa:
- C?t ch? h?p r?i n?i t?n-t?n ho?c ghp m?t ?ng ??ng m?ch nhn t?o.

- Lm ph?u thu?t b?c c?u n?i qua ch? h?p: ph?u thu?t ny lm t?t khi tu?i b?nh nhn t? 8-14.
Sau ph?u thu?t hay b? t?ng huy?t p th? pht, vim ??ng m?ch ch?u, ??ng m?ch tu?n hon bn d? b? t?c.
9. T? ch?ng Fallot (Tetralogy of Fallot).
9.1. ??i c??ng v phn lo?i:
+ L lo?i b?nh tim b?m sinh c tm, chi?m kho?ng 10% t?ng s? b?nh nhn c b?nh tim b?m sinh.
+ T? ch?ng Fallot bao g?m cc b?nh:
- Thng lin th?t: hay g?p ? ph?n mng ho?c ph?n c? ti?p xc v?i ph?n mng c?a vch lin th?t.
- H?p d??i van ??ng m?ch ph?i do ph ??i ph?n ph?u, t khi h?p ??ng m?ch ph?i ? cc v? tr khc.
- Ph ??i th?t ph?i do h?u qu? c?a h?p d??i van ??ng m?ch ph?i.
- ??ng m?ch ch? c??i ln vch lin th?t.
?i khi cn k?t h?p v?i cc d? t?t b?m sinh khc nh?:
. ??ng m?ch ch? xu?t pht t? th?t ph?i.
. Thng lin nh? tp 1 ho?c 2, g?i l ng? ch?ng Fallot (Pentology).
. Th?t ph?i 2 bu?ng.
. Khng c van ??ng m?ch ph?i ho?c thi?u s?n ??ng m?ch ph?i.
+ Phn lo?i: d?a theo m?c ?? p l?c th?t ph?i v th?t tri, m?c h?p t?c ph?n t?ng mu c?a th?t ph?i v thng lin th?t, ng??i ta chia lm 5 lo?i:
- Fallot n?ng: c thi?u s?n van ??ng m?ch ph?i, dng mu ln ph?i ph? thu?c vo tu?n hon bn c?a m?ch mu ph? qu?n ho?c t?n t?i ?ng ??ng m?ch.
- Fallot kinh ?i?n: c shunt t? ph?i qua tri, bi?u hi?n tm ti n?ng, ngn tay di tr?ng.
- Fallot m?c ?? trung bnh: shunt 2 chi?u, th??ng khng c tm khi ngh? ng?i, m?ch mu ph?i bnh th??ng.
- Fallot c thng lin th?t chi?m ?u th?: do h?p ??ng m?ch ph?i nh? nn shunt tri qua ph?i chi?m ?u th? qua l? thng lin th?t, b?nh ko di v?i suy tim m?n tnh.
- Fallot v?i h?p ??ng m?ch ph?i chi?m ?u th?: l? thng lin th?t nh?, ??ng m?ch ph?i h?p n?ng, p l?c ??ng m?ch ph?i t?ng cao g?n b?ng p l?c ??ng m?ch ngo?i vi.
9.2. B?nh sinh:
B?nh ph? thu?c vo 2 y?u t?.
- Kch th??c c?a l? thng lin th?t.
- M?c ?? t?c h?p c?a ??ng m?ch ph?i.
Khi l? thng lin th?t l?n, p l?c th?t tri v th?t ph?i cn b?ng nhau; n?u h?p ??ng m?ch ph?i khng nhi?u th p l?c th?t ph?i khng cao h?n p l?c ??ng m?ch ngo?i vi gy shunt 2 chi?u:
. Shunt tri qua ph?i ? th?i k? th?t co ??ng th? tch v th?i k? tm tr??ng.
. Shunt ph?i qua tri ? th?i k? tm thu.
9.3. Tri?u ch?ng lm sng v c?n lm sng:
9.3.1. Tri?u ch?ng lm sng:
- C? th? ch?m pht tri?n, chng m?t m?i khi v?n ??ng th? l?c, d? b? ng?t, l?m, tm ti, ?i khi b? co gi?t do thi?u oxy no.
- Tm: c khi tm ngay sau khi sinh, c khi sau vi thng ho?c ? tu?i thi?u nin. Khi c kh th? th ph?i ng?i x?m th ?? (do lc ? ??ng m?ch ph?i ?? b? co th?t h?n nn gi?m dng shunt ph?i qua tri).
- Ngn tay di tr?ng xu?t hi?n s?m, c khi c t? khi tr? d??i 1 tu?i, tri?u ch?ng ny r v n?ng h?n ? tu?i thi?u nin.
- L?ng ng?c bi?n d?ng.
- T?nh m?ch c? n?i v ??p theo nh?p tim.
- Nghe th?y ti?ng th?i tm thu m?nh ? lin s??n II-III d?c b? tri x??ng ?c do h?p ??ng m?ch ph?i v ti?ng th?i tm thu m?nh ? lin s??n III-IV c?nh ?c tri do thng lin th?t, T2 m? ho?c m?t. Rung miu tm thu t?i cc vng nghe tim trn.
- Th?t ph?i to v ??p m?nh ? vng th??ng v? [Hartzer (+)].
9.3.2. Tri?u ch?ng c?n lm sng:
- T?ng h?ng c?u (6-10 tri?u/mm3), t?ng hematocrit 60-70%.
- X quang: th?t ph?i to ra, cung ??ng m?ch ph?i x?p, ph?i sng, ??ng m?ch ch? gin.
- ?i?n tim: tr?c ph?i, ph ??i th?t ph?i m?nh, gin nh? ph?i.
- Siu m: c th? th?y ???c l? thng lin th?t, vch lin th?t v?n ??ng ngh?ch th??ng; ph ??i ph?n t?ng mu c?a ??ng m?ch ph?i; thnh th?t ph?i ph ??i v kch th??c th?t ph?i th tm tr??ng l?n; nhn ???c dng mu qua l? thng lin th?t; th?y ??ng m?ch ch? c??i ln vch lin th?t.
- Thng tim: c gi tr? ch?n ?on xc ??nh.
. p l?c th?t ph?i b?ng th?t tri, t?ng chnh p gi?a th?t ph?i v ??ng m?ch ph?i.
. Gi?m ?? bo ho xy ? th?t tri.

. ?ng thng tim ph?i sang ???c th?t tri v ln ???c ??ng m?ch ch? qua l? thng lin th?t.
. Ch?p bu?ng th?t ph?i c?n quang th?y thu?c sang c? th?t tri v ln ??ng m?ch ch?, th?y ???c c? ch? h?p ??ng m?ch ph?i.
9.4. Ch?n ?on:
9.4.1. Ch?n ?on xc ??nh:
- Lm sng: tm, ngn tay di tr?ng, ti?ng th?i tm thu v rung miu tm thu ? lin s??n III- IV c?nh ?c tri.
- X quang: gin th?t ph?i.
- ?i?n tim: dy th?t ph?i m?nh.
- Siu m: th?y hnh ?nh c?a t? ch?ng Fallot.
- Thng tim cho ch?n ?on xc ??nh.
9.4.2. Ch?n ?on phn bi?t:
- V?i b?nh tim b?m sinh c tm:
. Thng lin nh?, thng lin th?t ? c ??o shunt .
. ??o g?c ??ng m?ch: hi?m g?p, th??ng ch?t ? tu?i nh?.
. Ebstein: van 3 l h? th?p so v?i van 2 l, X quang tim to.
- V?i tam ch?ng Fallot g?m: h?p ??ng m?ch ph?i, ph ??i th?t ph?i v thng lin nh?.
- V?i ng? ch?ng Fallot: t? ch?ng Fallot k?t h?p v?i thng lin nh?.
9.5. ?i?u tr?:
9.5.1. ?i?u tr? n?i khoa:
- Phng ch?ng vim mng trong tim nhi?m khu?n.
- ?i?u tr? nh?ng c?n thi?u xy k?ch pht b?ng cho ng?i x?m hay t? th? g?i-ng?c, th? xy, tim morphin b?p th?t.
- Dng thu?c ?c ch? bta ?? gi?m chnh p gi?a th?t ph?i v ??ng m?ch ph?i: propranolol 40 mg x 1vin/ngy.
- Thu?c ch?ng ?ng, ng?n ng?ng k?t ti?u c?u ?? phng ch?ng t?c m?ch: aspirin 0,1- 0,25 g x 1 gi/ngy, u?ng lc no.
9.5.2. ?i?u tr? ngo?i khoa:
S?a ch?a cc d? t?t c trong t? ch?ng Fallot. Nn m? s?m khi tr? d??i 5 tu?i. T? l? t? vong khi m? cn cao t?i 10-25%.

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