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Hướng dẫn của ESC 2022 về quản lý bệnh nhân rối loạn nhịp thất và phòng ngừa đột tử do tim – P9

TS. PHẠM HỮU VĂN

 

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7.1.6. Bệnh cơ tim tâm sinh

Những tiến bộ trong phẫu thuật sửa chữa và điều trị y tế đã cải thiện tiên lượng lâu dài của trẻ em sinh ra mắc bệnh tim bẩm sinh (CHD). Hơn 90% hiện sống sót đến tuổi trưởng thành, 882 và do đó tỷ lệ mắc bệnh CHD ở người trưởng thành ngày càng tăng. [883] Với ít bệnh nhân tử vong do các biến cố chu phẫu và suy tim sớm hơn, SCD đã trở thành nguyên nhân gây tử vong hàng đầu ở người lớn mắc bệnh CHD đã được sửa chữa. [884] Sự kết hợp của các vết mổ phẫu thuật, sẹo cơ tim và các bất thường về giải phẫu còn sót lại hoặc mới tạo thành nền tảng cho VA (Hình 26).

Phân tầng nguy cơ SCD ở bệnh nhân CHD và không có VA dai dẳng được ghi nhận vẫn còn khó khăn do nhóm bệnh nhân hỗn hợp, không có RCT, và các nghiên cứu quan sát tương đối nhỏ. Ở những bệnh nhân có sinh lý hai thất và LV hệ thống, tiêu chuẩn chuẩn của LVEF ≤ 35% được sử dụng để lựa chọn bệnh nhân cấy ICD phòng ngừa tiên phát. [885,886] Đối với những bệnh nhân ngất không giải thích được, các triệu chứng rối loạn nhịp tim nghiêm trọng như đánh trống ngực hoặc tiền ngất và NSVT, nên xem xét PES. [887] Ở những bệnh nhân không có triệu chứng với tứ chứng Fallot (TOF) nhưng có các chỉ số khác về chất nền VA, đánh giá điện sinh lý có thể tinh chỉnh phân tầng nguy cơ. [888] Thời đại phẫu thuật và các kỹ thuật được sử dụng ảnh hưởng đến chất nền VA và sự xuất hiện của VA và cần được xem xét. Ví dụ, việc sử dụng miếng vá sửa chữa xuyên hình khuyên ở bệnh nhân TOF được phát hiện có liên quan đến nguy cơ mắc VA thấp hơn. [889] Lợi ích của điều trị ICD phòng ngừa tiên phát ở bệnh nhân có RV đơn độc hoặc hệ thống không có bệnh được xác lập rõ ràng và yêu cầu xem xét bệnh – và các yếu tố cụ thể của bệnh nhân. [890,891]

Ở những bệnh nhân CHD có VA dai dẳng hoặc sống sau CA, việc đánh giá toàn diện các yếu tố kích hoạt, gồm hình ảnh tim (đặc biệt là CMR) và đánh giá huyết động là rất quan trọng (Hình 26). [892] Nếu phát hiện các bất thường về giải phẫu đã có từ trước hoặc mới đòi hỏi cần can thiệp, kế hoạch điều trị nên xem xét việc lập bản đồ và cắt ngang chất nền VT trước khi phẫu thuật, vì khả năng tiếp cận có thể bị suy giảm sau phẫu thuật. [888,893,894]

Đánh giá và can thiệp trước phẫu thuật đặc biệt quan trọng ở những bệnh nhân TOF trải qua phẫu thuật định giá lại van phổi. Ở những bệnh nhân được chọn mắc CHD (bao gồm cả những người được sửa chữa vách liên nhĩ để chuyển vị trí của các động mạch lớn, phẫu thuật Fontan và dị thường Ebstein), đánh giá và điều trị SVT (như nhịp nhanh vào lại nhĩ hoặc nhịp nhanh vào lại AV) với dẫn truyền thất nhanh cũng nên được xem xét. [890,895,896]

Trong trường hợp không thể xác định có các yếu tố có thể đảo ngược được, cấy ICD để phòng ngừa SCD thứ phát được khuyến cáo. [349,350] Trong khi hệ thống ICD qua tĩnh mạch được sử dụng thường xuyên nhất và có ưu điểm chống nhịp tim nhanh và chống nhịp tim chậm, ICD dưới da có thể thay thế ở những bệnh nhân được chọn có đường tiếp cận tĩnh mạch hạn chế đến tâm thất hoặc có shunt trong tim.

Ở bệnh nhân CHD, MVT hầu hết xuất hiện do nhịp nhanh vào lại sử dụng các eo giải phẫu được bao bọc bởi van, vật liệu vá và vết mổ. Các nghiên cứu lập bản đồ và triệt phá sớm, đặc biệt ở bệnh nhân TOF, đã xác định các eo giải phẫu quan trọng của vị trí giải phẫu có thể tái tạo được. [897,898] Theo khái niệm này, các eo giải phẫu đó có thể được tái tạo và cắt ngang ở nhịp xoang trong quá trình triệt phá qua catheter bằng hệ thống lập bản đồ điện giải phẫu, đạt tỷ lệ thành công cấp tính là 80%. [888,899–901] Việc sử dụng block dẫn truyền qua các eo giải phẫu như một điểm cuối của thủ thuật, ngoài khả năng tạo ra VT, đã cải thiện hơn nữa kết quả triệt phá lâu dài. [888,899]

Theo đó, triệt phá qua ống thông được khuyến cáo đặc biệt ở những bệnh nhân TOF có SMVT tái phát. Do sự phức tạp của bệnh nhân CHD cũng như chất nền của VT, các thủ thuật đó nên được thực hiện tại các trung tâm có chuyên môn về triệt phá bệnh nhân CHD qua catheter. Triệt phá qua catheter có thể được xem xét thay cho cấy ICD ở những bệnh nhân TOF chọn lọc có SMVT và chức năng hai tâm thất được bảo tồn, cung cấp kết quả sau thủ thuật kết hợp của không thể tạo ra và block dẫn truyền qua eo giải phẫu có thể đạt được. [888,899]

Bảng khuyến cáo 38—Các khuyến cáo dành cho phân tầng nguy cơ và phòng ngừa đột tử tim tiên phát do bệnh tim bẩm sinh

Các khuyến cáo Classa Levelb
Phân tầng nguy cơ và ngăn ngừa SCD tiên phát
Tất cả các bệnh nhân CHD
Ở người lớn mắc CHD có sinh lý hai tâm thất và tâm thất trái có biểu hiện suy tim có triệu chứng (NYHAII/III) và EF ≤35% mặc dù OMT ≥ 3 tháng, cấy ICD được chỉ định. [885,886]  

I

 

C

Ở những bệnh nhân mắc CHD được cho là ngất do loạn nhịp và có rối loạn chức năng tâm thất ít nhất ở mức độ trung bình hoặc SMVT có thể được tạo ra trên PES, việc cấy ICD nên được xem xét. [887,889,902]  

IIa

 

C

Ở những bệnh nhân có rối loạn chức năng tâm thất đơn hoặc RV hệ thống tiến triển với các yếu tố nguy cơ bổ sung c, cấy ICD có thể được xem xét. [890,891]  

IIb

 

C

Tứ chứng Fallot
Ở những bệnh nhân sau khi sửa chữa TOF có triệu chứng rối loạn nhịp tim và NSVT, nên xem xét đánh giá điện sinh lý gồm PES. [889,903–905]  

IIa

 

C

Ở những bệnh nhân sau khi sửa chữa TOF có triệu chứng rối loạn nhịp tim và PES dương tính, hoặc kết hợp các yếu tố nguy cơ khác d và PES dương tính, nên xem xét cấy ICD.  

IIa

 

C

Ở những bệnh nhân sau khi sửa chữa TOF không có triệu chứng rối loạn nhịp tim, nhưng có sự kết hợp của các yếu tố nguy cơ khác, có thể xem xét đánh giá điện sinh lý, gồm PES.  

IIb

 

C

Ở những bệnh nhân TOF đã được sửa chữa trải qua phẫu thuật hoặc thay van phổi qua da, việc lập bản đồ qua catheter trước phẫu thuật và cắt ngang các eo giải phẫu liên quan đến VT trước hoặc trong khi can thiệp có thể được xem xét. [894]  

IIb

 

C

AV: nhĩ thất; CHD: bệnh tim bẩm sinh; CMR: cộng hưởng từ tim; EF: phân suất tống máu; ICD: máy khử rung tim có thể cấy; LV: van trái; NSVT: nhịp nhanh thất đơn hình tạm thời; NYHA: Hiệp hội Tim mạch New York; OMT: điều trị nội tối ưu; PES: kích thích điện được lập trình; RV: thất phải; SCD: đột tử do tim; SMVT: nhịp nhanh thất đơn hình dai dẳng; TOF: tứ chứng Fallot; VT: nhịp nhanh thất.

  a Class khuyến cáo.

  b Mức độ bằng chứng.

  c Dữ liệu rất ít và các yếu tố nguy cơ có thể đặc hiệu cho từng tổn thương, gồm VT tạm thời, NYHAII/III, hở van AV nặng và QRS rộng ≥ 140ms (chuyển vị đại động mạch).

  d Các yếu tố nguy cơ khác gồm rối loạn chức năng RV hoặc LV vừa phải, CMR sẹo RV rộng, [906,907] thời gian QRS ≥180ms [886,908] và QRS phân đoạn nghiêm trọng. [909,910]

Bảng khuyến cáo 39—Các khuyến cáo dành cho phòng ngừa đột tử tim thứ phát và điều trị rối loạn nhịp thất trong bệnh ti bẩm sinh

Các khuyến cáo Classa Levelb
Phân tầng nguy cơ và ngăn ngừa SCD tiên phát
Tất cả các bệnh nhân CHD
Ở những bệnh nhân CHD có VA dai dẳng, nên đánh giá các tổn thương còn sót lại hoặc các bất thường về cấu trúc mới. [892,893] I B
Ở những bệnh nhân CHD có VT không dung nạp / CA do VF được cứu thoát, cấy ICD được chỉ định sau khi loại trừ các nguyên nhân có thể đảo ngược. [349,350]  

I

 

C

Ở những bệnh nhân CHD và các cú sốc SMVT hoặc ICD tái phát, có triệu chứng đối với SMVT không thể kiểm soát được bằng điều trị nội khoa hoặc lập trình lại ICD, việc triệt phá qua catheter được thực hiện tại các trung tâm chuyên khoa nên được xem xét. c [899–901]  

IIa

 

C

Ở những bệnh nhân CHD được lựa chọn (gồm sửa chữa vách ngăn tâm nhĩ để chuyển vị trí các động mạch lớn, phẫu thuật Fontan và dị tật Ebstein) biểu hiện CA, nên xem xét đánh giá và điều trị SVT với dẫn truyền thất nhanh. [890,895]  

IIa

 

C

Tứ chứng Fallot
Ở những bệnh nhân TOF đã được sửa chữa có biểu hiện SMVT hoặc điều trị ICD thích hợp, có triệu chứng tái phát cho SMVT, việc triệt phá qua catheter được thực hiện ở các trung tâm chuyên khoa được khuyến cáo. [899–901]  

I

 

C

Ở những bệnh nhân TOF đã được sửa chữa với SMVT đang trải qua phẫu thuật hoặc thay van phổi qua da, nên xem xét lập bản đồ qua catheter trước phẫu thuật và cắt ngang các eo giải phẫu liên quan đến VT trước hoặc trong khi can thiệp. [888,893,894]  

IIa

 

C

Ở những bệnh nhân TOF đã được sửa chữa với chức năng hai tâm thất được bảo tồn và SMVT có triệu chứng, triệt phá qua catheter hoặc triệt phá bằng phẫu thuật đồng thời được thực hiện tại các trung tâm chuyên khoa có thể được coi là một phương pháp thay thế cho liệu pháp ICD. [899,901]  

IIb

 

C

CA: ngừng tim; CHD: bệnh tim bẩm sinh; ICD: máy khử rung tim có thể cấy; SCD: đột tử do tim; SMVT: nhịp nhanh thất đơn hình dai dẳng; SVT: nhịp tim nhanh trên thất; TOF: tứ chứng Fallot; VA: rối loạn nhịp thất; VF: rung thất; VT: nhịp nhanh thất.

  a Class khuyến cáo.

  b Mức độ bằng chứng.

  c Khuyến cáo cụ thể cho TOF (ClassI-B)

7.2. Bệnh điện học tiên phát

7.2.1. Rung thất nguyên phát

Chẩn đoán rung tâm thất nguyên phát (IVF) được thực hiện ở những người sống sót sau SCA, tốt nhất là có VF được ghi nhận, sau khi loại trừ các nguyên nhân về cấu trúc, bệnh kênh, chuyển hóa hoặc nhiễm độc. [135.222.911–913] Các xét nghiệm chẩn đoán gồm hóa học máu, ECG (bao gồm cả chuyển đạo cao), CT tim/chụp động mạch vành, đo từ xa/Holter, kiểm tra gắng sức, siêu âm tim, test chẹn kênh natri CMR (xem Phần 5.2.3, tình huống 3) . [135,222,911] Xét nghiệm di truyền đối với bệnh kênh và gen bệnh cơ tim có thể được xem xét với tỷ lệ đột biến là 3–17%. [249.914.915] Có thể xem xét đánh giá lâm sàng của các thành viên gia đình thế hệ thứ nhất, nhưng hiệu quả chẩn đoán thấp. Đặc biệt, tầm quan trọng của mẫu tái cực sớm (ERP) được phát hiện ở những người thân không có triệu chứng là không chắc chắn. [182.916]

Ở bệnh nhân IVF, cấy ICD làm giảm nguy cơ tử vong do rối loạn nhịp tim tới 68% so với amiodarone [352,917–921] (Hình 27). Các nghiên cứu quan sát với thời gian theo dõi trung bình 5–6 năm, 21,0–29,6% bệnh nhân IVF bị tái phát rối loạn nhịp tim, tỷ lệ sốc ICD hàng năm tương ứng là 3,6–5,7%, trong khi 4,5–17,5% gặp phải những cú sốc không phù hợp. [919–921] Isoproterenol, verapamil, hoặc quinidine đã được sử dụng để điều trị cấp thời tình trạng phóng điện ICD tái phát hoặc bão điện. [912,913,918,922–926] Trong một số nghiên cứu nhỏ, quinidine có hiệu quả cao trong việc giảm hoặc thậm chí ngăn ngừa khả năng gây rối loạn nhịp tim trong quá trình kích thích được lập trình. [923,924,926] Hơn nữa, một nghiên cứu hồi cứu ở 46 bệnh nhân cho thấy giảm số lần sốc ICD trung bình từ 7,5 trên mỗi bệnh nhân trong 2,9 năm xuống còn 0,9 cú sốc trên mỗi bệnh nhân trong 3,7 năm, với sự giảm các cơn bão tâm thất từ 36 xuống còn 3 sau khi bắt đầu dùng quinidine. [922] Ở những bệnh nhân có các đợt VF tái phát được kích hoạt do PVC tương tự không đáp ứng với điều trị nội khoa, triệt phá qua ống thông đã cho thấy thành công (Hình 28). [186.221.333.493.927–930] PVC phổ biến nhất có nguồn gốc từ hệ thống Purkinje và có thể bị loại bỏ với tỷ lệ thành công cấp thời cao là 87–100%. [186.221.333.493.927–930] Bản đồ giải phẫu điện chi tiết cũng có thể tiết lộ những thay đổi cấu trúc cục bộ (62,5% trong một nghiên cứu). [248]

Bảng khuyến cáo 40 — Khuyến cáo quản lý các bệnh nhân có rung thất nguyên phát

Các khuyến cáo Classa Levelb
Đánh giá chẩn đoán
Người ta khuyến cáo VF nguyên phát được chẩn đoán ở người sống sót sau SCA tốt nhất là có tài liệu về VF, sau khi loại trừ nguyên nhân cấu trúc, bệnh lý kênh, chuyển hóa hoặc nhiễm độc. [222,911]  

I

 

B

Xét nghiệm lâm sàng (tiền sử, ECG và ECG chuyển đạo trước tim cao, test gắng sức, siêu âm tim) của các thành viên gia đình thế hệ thứ nhất của bệnh nhân VF nguyên phát có thể được xem xét. [222,278,916]  

IIb

 

B

Ở những bệnh nhân VF nguyên phát, xét nghiệm di truyền các gen liên quan đến bệnh lý kênh và bệnh cơ tim có thể được xem xét. [249,278,914,915]  

IIb

 

B

Ngăn ngừa SCD thứ phát và điều trị VAs
Cấy ICD được khuyến cáo ở bệnh nhân

VF nguyên phát. [352,917–919]

I B
Truyền isoproterenol, verapamil hoặc quinidine để điều trị cấp tính cơn bão điện hoặc ICD phát sốc tái phát nên được xem xét trong VF nguyên phát. [912.913.918.922–924.926]  

IIa

 

C

Quinidine nên được xem xét cho điều trị kéo dài ngăn chặn cơn bão điện hoặc ICD phóng điện tái phát trong VF nguyên phát. [923,924]  

IIa

 

B

Việc triệt phá qua catheter do các nhà điện sinh lý có kinh nghiệm nên được xem xét ở bệnh nhân VF nguyên phát có các đợt tái phát

VF được kích hoạt bằng một PVC tương tự không đáp ứng với điều trị thuốc. [927–930]

 

IIa

 

C

ECG: điện tâm đồ; ICD: máy khử rung tim có thể cấy; PVC: phức bộ thất sớm; SCA: ngừng tim đột ngột; SCD: đột tử do tim; VA: rối loạn nhịp thất; VF: rung tâm thất.

a Class khuyến cáo.

b Mức độ bằng chứng.

Hình 27. Thuật toán cho quản lý các bệnh nhân có rung thất nguyên phát, ICD: máy khử rung tim có thể cấy; PVC: phức bộ thất sớm.

7.2.2. Hội chứng QT dài (gồm hội chứng QT dài mắc phải)

LQTS được đặc trưng bởi khoảng QT kéo dài và VA chủ yếu được kích hoạt bởi hoạt hóa adrenergic. Độ tuổi trung bình tại thời điểm trình bày là 14 tuổi. Tỷ lệ SCD hàng năm ở những bệnh nhân không có triệu chứng với LQTS không được điều trị đã được ước tính là dưới 0,5%, [82] trong khi nó tăng lên khoảng 5% ở những người có tiền sử ngất. [931]

Các biến thể hiếm ở 17 gen [932] có liên quan đến LQTS. Tuy nhiên, mối quan hệ nhân quả đối với một số gen được xác định đã bị nghi ngờ. [166] Những gen không thể chối cãi là gen gây ra LQT1, LQT2

và LQTS3: KCNQ1, KCNH2 và SCN5A, tương ứng với các yếu tố kích hoạt gen đặc hiệu là gắng sức (LQTS1), cảm xúc căng thẳng (LQTS2) và ngủ (LQTS3). Sàng lọc di truyền xác định đột biến ở 75% trường hợp LQTS và ba gen chính chiếm 90% các trường hợp có kiểu gen dương tính. [178] Các loại phụ của LQTS có thể được nhóm lại như sau:

(1) LQTS nhiễm sắc thể thường trội (tỷ lệ mắc: 1 trên 2500) không có biểu hiện ngoài tim.

(2) LQTS nhiễm sắc thể thường có biểu hiện ngoài tim, bao gồm:

(a) Hội chứng Andersen–Tawil (LQT7), ngày càng được coi là thực thể của chính nó. [933,934]

(b) Hội chứng Timothy (LQT8), đặc trưng bằng QT kéo dài, dị tật ngón tay, dị tật tim, rối loạn phổ tự kỷ và rối loạn hình thái. [935]

(3) LQTS nhiễm sắc thể thường lặn (Hội chứng Jervell và Lange–Nielsen), kết hợp kéo dài QT quá mức với điếc bẩm sinh. [936]

Nhóm chuyên gia này đã xác nhận các tiêu chuẩn chẩn đoán được đề xuất trong phiên bản trước của hướng dẫn: QTc ≥ 480 ms hoặc điểm nguy cơ LQTS > 3 [937] (Bảng 10) để chẩn đoán lâm sàng (Hình

29 và 30). Khi có ngất hoặc CA do loạn nhịp tim, QTc ≥ 460 ms là đủ để xem xét chẩn đoán LQTS. Một thách thức đối với bác sĩ lâm sàng là thiết lập khoảng QT ở bệnh nhân có phức bộ QRS rộng (ví dụ như khi có nhịp thất hoặc khiếm khuyết dẫn truyền thất). Trong trạng thái này, một công thức

đã được đề xuất điều chỉnh QT theo thời lượng QRS. [938] Trong khi đo QT, bệnh nhân di chuyển nhanh từ tư thế nằm sang tư thế đứng có thể hữu ích cho chẩn đoán LQTS. [232,939] Phép thử epinephrine không được khuyến khích như một công cụ chẩn đoán thông thường vì khả năng tái tạo còn khiêm tốn. [137] Bệnh nhân được chẩn đoán lâm sàng LQTS được khuyến cáo trải qua tư vấn và xét nghiệm di truyền tại các trung tâm chuyên khoa để được quản lý theo kiểu gen cụ thể và cho phép xác định những người thân có nguy cơ. Những người thân có đột biến nhưng không kéo dài khoảng QT vẫn được chẩn đoán mắc LQTS, vì họ có nguy cơ bị VA, mặc dù ít gặp hơn so với những bệnh nhân có kiểu hình dương tính. [940]

Tất cả bệnh nhân LQTS đều nhận được lời tư vấn về cách tránh hạ kali máu, dùng thuốc kéo dài QT và các yếu tố kích hoạt đặc hiệu kiểu gen. [941–943] Thuốc chẹn beta cũng được khuyên dùng ở tất cả bệnh nhân LQTS. Thuốc chẹn beta không chọn lọc nadolol và propranolol có hiệu quả cao hơn trong việc giảm nguy cơ rối loạn nhịp tim. [940,944–946] Các thông số lâm sàng, điện tâm đồ và di truyền nên được xem xét để ước tính nguy cơ cá thể. [82] Gần đây, phân tầng nguy cơ dựa trên khoảng thời gian QT và kiểu gen đã được tích hợp trong máy tính LQTS (máy tính Rủi ro LQTS 1-2-3). [947]

Tiện ích của xét nghiệm di truyền được minh họa bằng nhu cầu tránh các nguy cơ đặc hiệu về kiểu gen và bằng cách sử dụng mexiletine như một phương pháp điều trị đặc hiệu kiểu gen cho LQT3, giúp làm giảm độ dài khoảng QT và số lượng các biến cố loạn nhịp tim. [948] Cần lưu ý rằng các đột biến khác nhau ở SCN5A cho thấy các phản ứng khác nhau với mexiletine. Ví dụ, các đột biến chọn lọc được xác định ở những bệnh nhân không đáp ứng với mexiletine cho thấy đặc điểm điện sinh lý đặc biệt khi nghiên cứu in vitro. [949,950] Ngoài ra, trong trường hợp đột biến gây ra hội chứng chồng chéo, mexiletine không tạo ra chênh lên của đoạn ST, trong khi flecainide đã được báo cáo làm chênh lên.

Do vai trò chưa chắc chắn của thuốc chẹn beta trong LQT3, không có dấu hiệu nào về việc nên dùng mexiletine như một liệu pháp độc lập hay kết hợp với thuốc chẹn beta. Xem xét rằng một số đột biến có thể không đáp ứng với mexiletine, nên thực hiện test uống để xác minh rằng QTc rút ngắn 40 mili giây trước khi kê đơn điều trị kéo dài. [948]

Những người sống sót sau CA có nguy cơ tái phát cao, ngay cả khi dùng thuốc chẹn beta (14% trong vòng 5 năm điều trị), ủng hộ việc sử dụng ICD ở những người sống sót sau CA. [952] Hơn nữa, cấy ICD được chỉ định khi bệnh nhân bị ngất và/hoặc VA mặc dù điều trị bằng thuốc tối ưu, vì các biến cố ngất có liên quan đến nguy cơ tăng CA. [953,954] Phụ nữ mắc LQTS, đặc biệt là LQT2, có nguy cơ rối loạn nhịp tim cao hơn cả khi mang thai và đặc biệt là năm đầu tiên sau sinh. [955] Những người mang đột biến thầm lặng có nguy cơ mắc các biến cố về tim thấp nhưng không đáng kể và việc sử dụng thuốc chẹn beta nên được xem xét ở nhóm bệnh nhân này. [956]

Bóc bỏ thần kinh giao cảm tim trái (LCSD) được khuyến cáo cho các bệnh nhân có triệu chứng mặc dù dùng thuốc chẹn beta khi ICD bị chống chỉ định hoặc bị từ chối, hoặc cho người mang ICD trải qua nhiều cú sốc khi đang dùng thuốc chẹn beta. Điều này được hỗ trợ bằng bằng chứng cho thấy LCSD, đặc biệt khi được thực hiện với kỹ thuật hỗ trợ bằng video, an toàn và hiệu quả, [957] được bệnh nhân dung nạp tốt [958] và không có tác động tiêu cực đến hoạt động tim mạch. [959] Tuy nhiên, do các biến chứng xảy ra và một nửa số bệnh nhân gặp phải các biến cố đột ngột sau thủ thuật, LCSD không phải là giải pháp thay thế cho ICD cho những bệnh nhân có nguy cơ cao. [960] Liệu pháp ICD dự phòng ngoài OMT có thể được xem xét ở những bệnh nhân LQTS không có triệu chứng được xác định có nguy cơ cao theo công cụ tính nguy cơ LQTS 1-2-3. [947] PES không hữu ích cho việc phân tầng nguy cơ trong LQTS. [961]

Hình 31 minh họa thuật toán quản lý bệnh nhân mắc hội chứng QT dài.


Hình 29 Điện tâm đồ hội chứng QT kéo dài và nhịp nhanh thất xoắn đỉnh. (A) Đặc điểm ECG trong ba type chính LQTS. (B) Ví dụ về xoắn đỉnh ở bệnh nhân nam có đột biến SCN5A (c.1238C.A, p. A413E). LQT: QT dài.

Hình 30 Thay đổi nhanh trên điện tâm đồ khi đứng và sự thay đổi sóng T ở bệnh nhân mắc hội chứng QT dài. (A) ECG thay đổi trong quá trình kiểm tra tư thế đứng nhanh ở bệnh nhân nam LQTS có đột biến KCNH2 (p.S818L), nhịp tim tăng có liên quan đến việc điều chỉnh khoảng QT ít hơn. (B) Sự thay đổi sóng T ở bệnh nhân nam có đột biến CACNA1C (p. G406R).

Hình 31 Sơ đồ quản lý bệnh nhân mắc hội chứng QT kéo dài. ICD, máy khử rung tim cấy ghép; LCSD: bóc bỏ thần kinh giao cảm tim trái; LQT, QT dài; N, Không; VA, rối loạn nhịp thất; Y: có. a Khuyến cáo chung: tránh dùng các thuốc kéó dài khoảng QT (http://www.creditmeds.org), điều chỉnh các bất thường về điện giải (hạ kali máu, hạ magie máu và hạ canxi máu), tránh các yếu tố kích thích đặc hiệu kiểu gen cho rồi loạn nhịp (bơi cường độ cao trong LQT1, tiếp xúc với tiếng ồn lớn trong LQT2). b Thuốc chẹn beta được ưu tiên: nadolol và propranolol.

(Còn nữa)

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