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Khuyến cáo của Hội tim mạch học Việt Nam về chẩn đoán và điều trị bệnh cơ tim phì đại (2022) – P6

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9.2.3 Điều trị

Phân tầng nguy cơ

Các mô hình dự báo nguy cơ ở trẻ em BCTPĐ gần đây được đề xuất nhưng chưa được dùng rộng rãi trong thực hành lâm sàng. Phân tầng nguy cơ đột tử ở trẻ em nên kết hợp các yếu tố nguy cơ cụ thể vì nhiều yếu tố nguy cơ ở trẻ em khác với người lớn. Ví dụ như liên quan của dầy thất trái với nguy cơ đột tử, trẻ em không có ngưỡng điểm Z bề dầy vách liên thất chính xác là điểm ngưỡng giới hạn để chỉ định đặt máy phá rung (ICD) phòng ngừa tiên phát.

Một số yếu tố nguy cơ đã được ghi nhận bao gồm tuổi nhỏ khi biểu hiện bệnh, tiền sử gia đình đột tử, tiền sử ngất, nhịp nhanh thất, huyết áp giảm bất thường khi gắng sức cũng như sự hiện diện của đột biến gene troponin T,…(105,244). Yếu tố nguy cơ trên siêu âm tim cũng được ghi nhận là khi thành thất trái dầy lan tỏa và bề dầy cuối tâm trương trên 30 mm(259–261). Tỷ lệ E/e’ tăng cao ở trẻ em BCTPĐ cũng cho thấy liên quan với nguy cơ nhịp nhanh thất, ngưng tim hoặc tử vong cao hơn(262). Rối loạn chức năng tâm thu và dãn buồng tim cũng được biết là có liên quan suy tim và giảm sống còn.

Các vấn đề điều trị

Nguyên tắc điều trị

Mục tiêu là giảm triệu chứng, giảm chênh áp qua đường thoát thất, bảo tồn chức năng thất trái và kéo dài sống còn(246). Điều quan trọng là, các phương pháp điều trị không được chứng minh là có thể làm thay đổi sự tiến triển của bệnh. Các chiến lược điều trị tập trung vào việc cải thiện triệu chứng, sử dụng máy phá rung cấy được (ICD) ở những bệnh nhân được đánh giá là có nguy cơ đột tử do tim cao.

Thuốc

Các loại thuốc được sử dụng để điều trị BCTPĐ nhằm mục đích giảm tắc nghẽn đường ra thất trái, giảm nhu cầu oxy cơ tim và làm chậm nhịp tim để cải thiện đổ đầy thất. Thuốc chẹn beta được sử dụng phổ biến nhất ở trẻ em.

Tránh các tình huống làm giảm thể tích. Giảm thể tích nhát bóp làm nặng thêm ảnh hưởng của hẹp đường thoát vì dẫn đến hạ huyết áp, choáng váng và ngất.

Tránh dùng các thuốc làm tăng hẹp đường thoát, như là thuốc dãn mạch (thuốc ức chế men chuyển / ức chế thụ thể, thuốc ức chế kênh calci DHP và nitroglycerin) làm giảm sức cản ngoại biên, tăng tắc nghẽn đường ra thất trái và áp lực đổ đầy thất vì thế gây hạ huyết áp và tăng suy tim.

Thuốc lợi tiểu làm giảm thể tích đổ đầy, thất trái nhỏ hơn và tắc nghẽn đường thoát tăng hơn. Tuy nhiên, cân nhắc sử dụng thuốc lợi tiểu ở bệnh nhân BCTPĐ không tắc nghẽn với suy tim kéo dài và quá tải thể tích.

Digoxin: thường tránh dùng ở BCTPĐ do thuốc làm tăng sức bóp cơ tim. Tuy nhiên, những bệnh nhân BCTPĐ giai đoạn sau đã bị rối loạn chức năng tâm thu là ngoại lệ. Ở những trường hợp  này, điều trị suy tim tiêu chuẩn có thể được chỉ định dù là BCTPĐ và chức năng tâm thu thất trái bảo tồn.

Điều trị hẹp đường ra thất trái

 Điều trị giảm bề dầy vách thất được chỉ định ở bệnh nhân có triệu chứng, kháng trị với thuốc kể cả khi nghỉ hay khi gắng sức mà chênh áp qua đường thoát thất trên 50 mmHg. Phương pháp được chọn lựa ở trẻ em là phẫu thuật cắt vách liên thất. Phẫu thuật được khuyến cáo thực hiện ở trung tâm chuyên sâu về BCTPĐ với đội ngũ có kinh nghiệm(263).

Điều trị rối loạn nhịp và ngăn ngừa đột tử do tim

Máy phá rung cấy được (ICD) là liệu pháp tốt nhất hiện có cho bệnh nhân BCTPĐ cứu sống sau đột tử do tim hoặc những người có nguy cơ cao bị loạn nhịp thất và đột tử. Do nguy cơ liên quan đến dây dẫn được đặt khi trẻ lớn nhanh nên tuổi đặt thường ở tuổi vị thành niên, khi kích cỡ cơ thể đã gần như người lớn. Chỉ định ở tuổi nhỏ hơn nếu có nhiều yếu tố khác hoặc nguy cơ đột tử rất cao.

Thuốc kiểm soát nhịp tim ở trẻ em cần được dùng khi trẻ chưa có chỉ định ICD mà xuất hiện nhịp nhanh thất thường xuyên, phải sốc điện chuyển nhịp rung thất / nhịp nhanh thất.

Đặt ICD

Quyết định đặt ICD dựa trên đánh giá cá thể hoá và xác định nguy cơ tuỳ thuộc tuổi, lâm sàng tổng thể, biến chứng liên quan đến việc đặt máy bao gồm cả vấn đề tâm lý trẻ khi đeo máy và sốc ICD không thích hợp.

Các yếu tố nguy cơ gây đột tử do tim – Tỷ lệ rối loạn nhịp tim hoặc đột tử đe dọa tính mạng được báo cáo ở bệnh nhi BCTPĐ dao động từ dưới 1 đến 2,8% sau một năm chẩn đoán và 9% sau 5 năm sau chẩn đoán(92).

Đối với trẻ em BCTPĐ, cũng cần quan tâm một số vấn đề như vị trí dây điện cực được đặt ở thượng tâm mạc đối với các trẻ dưới 30kg và những trẻ em cần đặt dụng cụ tái đồng bộ thất trái. Các biến chứng của ICD có thể cao hơn ở trẻ em và thanh thiếu niên vì nhịp tim cơ bản cao làm tăng nguy cơ máy sốc không thích hợp, tăng trưởng thể chất dẫn đến tăng nguy cơ gãy dây điện cực, cũng như nhu cầu thay thế, lấy dụng cụ ra nhiều lần trong đời. Ở những bệnh nhân nhỏ hơn, đặt máy đường tĩnh mạch có thể dẫn đến nguy cơ thất bại cao hơn những bệnh nhân lớn. Đặt ICD dưới da ở những bệnh nhân nhỏ còn tăng nguy cơ biến chứng, bao gồm hở dụng cụ ra da do xói mòn.

Ghép tim: Là lựa chọn cuối cùng trong điều trị. Tuy nhiên, vấn đề về tạng ghép cũng như vấn đề ghép tim cho trẻ em tại Việt Nam vẫn còn nhiều hạn chế.

Những chăm sóc khác: Các khía cạnh quan trọng khác trong chăm sóc sức khỏe lâu dài ở trẻ em mắc BCTPĐ cần được quan tâm.

Chủng ngừa: Trẻ em mắc BCTPĐ phải được chủng ngừa đủ các loại vaccine, bao gồm cả vaccine ngừa phế cầu và cúm hàng năm.

Theo dõi thường xuyên các thông số tăng trưởng – cũng cần theo dõi sự tăng trưởng và phát triển ở trẻ em BCTPĐ như tất cả trẻ khác. Đối với sơ sinh và trẻ em BCTPĐ trong quá trình theo dõi, ghi nhận chậm phát triển về thể chất có thể là dấu hiệu chính của suy tim.

Khám định kỳ: Theo dõi và đánh giá các triệu chứng của hẹp đường ra thất trái và suy tim. Nếu bệnh nhân xuất hiện các triệu chứng mới hoặc triệu chứng nặng hơn như đau ngực, tiền ngất / ngất, đánh trống ngực hoặc các triệu chứng suy tim cần được chuyển ngay đến bác sĩ chuyên khoa tim mạch nhi để đánh giá.

Thể dục thể thao: liên quan đến nguy cơ đột tử do tim khi gắng sức, cần chọn lựa hoạt động thể thao phù hợp cho bệnh nhân BCTPĐ. Đồng thuận trong các khuyến cáo, bệnh nhân đã xác định hoặc nghi ngờ BCTPĐ không nên tham gia các môn thể thao đối kháng, trừ các môn cường độ thấp (ví dụ: Golf, Bida, Bowling, Bóng chày,…)(229). Cần giải thích rõ về bệnh, nguy cơ và lợi ích khi tham gia thể thao ở các bệnh nhân này. Những bệnh nhân BCTPĐ có ghi nhận bất thường gene nhưng không biểu hiện bệnh có thể tham gia vào các môn thể thao cạnh tranh cường độ cao hơn(62).

Phẫu thuật ngoài tim: Bệnh nhi có BCTPĐ có hẹp đường ra thất trái sẽ có nhiều nguy cơ hơn khi phẫu thuật hoặc các thủ thuật cần gây mê. Cần lên kế hoạch cụ thể khi cần phẫu thuật ngoài tim bao gồm hội chẩn giữa bác sĩ tim mạch nhi với bác sĩ gây mê và hậu phẫu.

Sàng lọc và theo dõi thành viên gia đình

Khuyến cáo 2020 của ACC / AHA(26):

Sàng lọc trong gia đình: Đối với người BCTPĐ có xét nghiệm gene có đột biến đều cần kiểm tra siêu âm tim của người thân thế hệ thứ nhất. Lập kế hoạch kiểm tra siêu âm tim và theo dõi lâm sàng 1 – 3 năm một lần vì BCTPĐ có thể biểu hiện ở mọi lứa tuổi trong gia đình.

Sàng lọc di truyền: Tư vấn thực hiện xét nghiệm di truyền với bất cứ trường hợp nào BCTPĐ. Khi phát hiện biến thể gây bệnh, xét nghiệm di truyền thực hiện cho tất cả những người thân thế hệ thứ nhất song song với theo dõi lâm sàng liên tục ở những người thân mang biến thể gây bệnh. Khi BCTPĐ không phát hiện được biến thể gây bệnh, xét nghiệm cho thân nhân là không hữu ích. Điều quan trọng là phải đánh giá lại khả năng gây bệnh của biến thể sau mỗi 2 – 3 năm vì biểu hiện có thể phát triển theo thời gian và thời điểm cũng như cách thức sàng lọc cho thành viên trong gia đình có thể thay đổi theo.

Bảng 9. Thực hiện tầm soát người thân trực hệ không triệu chứng của bệnh nhân BCTPĐ bằng điện tâm đồ và siêu âm tim 2D (26)

Tuổi thành viên thế hệ thứ nhất Lần sàng lọc đầu tiên Lặp lại ECG, siêu âm
Trẻ em và vị thành niên từ gia đình có xét nghiệm gene dương tính và gia đình với bệnh khởi phát sớm Ở thời điểm chẩn đoán thành viên khác trong gia đình BCTPĐ Mỗi 1 – 2 năm
Tất cả trẻ em và vị thành niên Ở thời điểm sau khi chẩn đoán thành viên khác trong gia đình BCTPĐ nhưng không trễ quá tuổi dậy thì. Mỗi 2 – 3 năm
ECG, Electrocardiogram, Điện tâm đồ

 

10. Ra quyết định

Loại MCC Khuyến cáo về ra quyết định
1 B 1. Đối với bệnh nhân BCTPĐ hoặc nguy cơ mắc BCTPĐ, những quyết định liên quan đến kế hoạch chăm sóc (như đánh giá di truyền, mức độ hoạt động, lối sống và chọn lựa điều trị) cần được trao đổi với bệnh nhân và thân nhân của họ kể cả việc đề cập đến nguy cơ, lợi ích, cơ hội và hậu quả của từng phương pháp điều trị (264–269).

Việc trao đổi đầy đủ giữa bác sĩ và bệnh nhân cũng như thân nhân của họ giúp quyết định liên quan đến chăm sóc bệnh nhân BCTPĐ được chọn lựa và thực hiện một cách phù hợp và hiệu quả nhất cho bệnh nhân BCTPĐ.

11. Trung tâm Bệnh cơ tim phì đại đa ngành

Loại MCC Khuyến cáo về Trung tâm Bệnh cơ tim phì đại đa ngành
1 C 1. Ở những bệnh nhân BCTPĐ, điều trị cắt vách nên được thực hiện ở những trung tâm có kinh nghiệm (Bảng 3 và Bảng 10).
2a C 2. Ở những bệnh nhân BCTPĐ liên quan đến những quyết định điều trị bệnh phức tạp, việc hội chẩn hoặc chuyển bệnh nhân đến những trung tâm điều trị BCTPĐ toàn diện hoặc sơ cấp là hợp lý (Bảng 10).

Trung tâm BCTPĐ được xem là có kinh nghiệm khi trung tâm có những chuyên gia có khả năng điều trị BCTPĐ bao gồm nội khoa, thủ thuật và phẫu thuật cùng với số lượng bệnh nhân đủ lớn và các số liệu thống kê cho thấy kết cục lâm sàng tốt.

Trung tâm BCTPĐ sơ cấp đa ngành có một đội ngũ có khả năng cao trong việc điều trị bệnh nhân BCTPĐ, bao gồm những kỹ năng được đề cập trong Bảng 10.

Bảng 10. Khả năng được đề nghị của một trung tâm BCTPĐ sơ cấp và toàn diện

Khả năng chăm sóc BCTPĐ Trung tâm BCTPĐ toàn diện Trung tâm BCTPĐ

sơ cấp

 Trung tâm

giới thiệu bệnh

/bác sĩ
Chẩn đoán x x x
Siêu âm tim qua thành ngực ban đầu và theo dõi x x x
Hình ảnh học siêu âm tim nâng cao phát hiện nghẽn đường ra thất trái tiềm ẩn x x  
Siêu âm tim hướng dẫn điều trị giảm bề dầy vách liên thất x *  
Cộng hưởng từ tim chẩn đoán và phân tầng nguy cơ x x  
Đánh giá đường ra thất trái xâm nhập x x *
Chụp động mạch vành x x x
Trắc nghiệm gắng sức phát hiện nghẽn đường ra thất trái hoặc đánh giá điều trị suy tim rất nặng/ghép tim x x  
Tư vấn và thực hiện tầm soát gia đình (hình ảnh học và di truyền) x x x
Tư vấn / xét nghiệm di truyền x x *
Đánh giá nguy cơ đột tử x x x
Quyết định đặt ICD loại 1 và 2a ở bệnh nhân người lớn x x x
Quyết định đặt ICD loại 2b ở bệnh nhân người lớn x x  
Đặt ICD (người lớn) x x *
Quyết định và đặt ICD ở bệnh nhân trẻ em / thiếu niên x *  
Quản lý rung nhĩ ban đầu và phòng ngừa đột quỵ x x x
Cắt đốt rung nhĩ qua ống thông x x *
Quản lý ban đầu suy tim phân suất tống máu giảm và bảo tồn x x x
Điều trị suy tim rất nặng (ghép tim, CRT) x *  
Điều trị bằng thuốc BCTPĐ tắc nghẽn có triệu chứng x x x
Điều trị xâm nhập BCTPĐ tắc nghẽn có triệu chứng   ┼  
Tư vấn lựa chọn nghề nghiệp và lối sống khỏe mạnh khác với hoạt động cường độ mạnh hoặc có tính cạnh tranh x x x
Các chọn lựa tư vấn về việc tham gia thi đấu các môn thể thao cường độ mạnh hoặc có tính cạnh tranh x x  
Điều trị bệnh nhân nữ BCTPĐ mang thai x *  
Điều trị bệnh đồng mắc x x x
* Tùy thuộc vào khả năng của viện

┼ Nếu thủ thuật này được thực hiện, cần chứng minh kết cục tương đương với trung tâm toàn diện

CRT, Cardiac Resynchronization Therapy, Điều trị tái đồng bộ tim; ICD, Implantable Cardioverter-Defibrillator, Máy phá rung cấy được.

 Bảng 11. Mục tiêu cần đạt được của điều trị giảm bề dầy vách liên thất xâm nhập

  Tỷ lệ
Cắt vách Đốt cồn
Tử vong 30 ngày ≤ 1% ≤ 1%
Biến chứng 30 ngày (chèn ép tim cấp, bóc tách động mạch liên thất trước, nhiễm trùng, chảy máu nghiêm trọng) ≤ 10% ≤ 10%
Nghẽn dẫn truyền tim hoàn toàn cần đặt máy tạo nhịp vĩnh viễn ≤ 5% ≤ 10%
Thay van hai lá trong vòng 1 năm ≤ 5%  
Hở van hai lá tồn lưu nặng ≤ 5% ≤ 5%
Lặp lại thủ thuật ≤ 3% ≤ 10%
Cải thiện phân độ NYHA > 90% > 90%
Chênh áp qua đường ra thất trái dưới 50 mmHg lúc nghỉ và lúc làm trắc nghiệm phát hiện nghẽn đường ra thất trái tiềm ẩn > 90% > 90%

 

TÀI LIỆU THAM KHẢO

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  2. Marian AJ, Braunwald E. Hypertrophic Cardiomyopathy. Circulation Research. 2017;121(7):749-770. https://doi.org/10.1161/CIRCRESAHA.117.311059
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