Khuyến cáo của Liên chi hội Tim mạch Tp. Hồ Chí Minh về chẩn đoán và điều trị tăng áp phổi

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PGS.TS.BS HỒ HUỲNH QUANG TRÍ

Tham gia biên soạn:

BSCKII. LÊ THỊ ĐẸP, ThS.BSCKII. TRẦN THỊ TUYẾT LAN,

ThS.BSCKII. HUỲNH THANH KIỀU, ThS.BS. PHẠM ĐỖ ANH THƯ,

BSCKI. VŨ NĂNG PHÚC, BSCKI. PHẠM THỤC MINH THỦY

Biên tập: TRẦN THỊ THANH NGA

DANH MỤC TỪ VIẾT TẮT

1. Đại cương

Tăng áp phổi (TAP) là một rối loạn sinh lý bệnh liên quan đến nhiều điều kiện lâm sàng và có thể kết hợp với nhiều bệnh tim mạch và hô hấp.

Trong những năm gần đây, tiến bộ về nhận thức lâm sàng và phương tiện cận lâm sàng giúp phát hiện, phân loại theo nguyên nhân và điều trị TAP hiệu quả hơn. Nhờ đó chất lượng cuộc sống và tuổi thọ bệnh nhân có thể tăng thêm.

Khuyến cáo này đề cập đến tất cả các khía cạnh của TAP, giúp chẩn đoán và điều trị thích hợp; có nhấn mạnh đến hai thể bệnh tăng áp động mạch phổi (PAH) và tăng áp phổi do huyết khối tĩnh mạch thuyên tắc (CTEPH: Chronic Thromboembolic Pulmonary Hypertension)

Bảng 1.1 Phân loại các khuyến cáo

Bảng 1.2 Các mức chứng cứ

2. Định nghĩa và phân loại

2.1. Định nghĩa

• Các định nghĩa về TAP dựa vào huyết động qua kết quả thông tim phải. Tuy vậy chẩn đoán sau cùng cần được thêm vào bệnh cảnh lâm sàng và các thăm khám cận lâm sàng khác.
• Tăng áp phổi được chẩn đoán khi áp lực trung bình ĐMP > 20 mmHg lúc nghỉ, qua thông tim. Sức cản mạch phổi bình thường khi dưới 2 đơn vị Woods. Áp lực bít mao mạch phổi bình thường khi dưới 15 mmHg. Cần phân biệt tăng áp động mạch phổi, tăng áp phổi trước mao mạch, tăng áp phổi sau mao mạch, tăng áp phổi phối hợp trước và sau mao mạch. Tăng áp phổi khi gắng sức được định nghĩa là tỷ lệ ALĐMP trung bình/ cung lượng tim lúc nghỉ và lúc gắng sức thay đổi > 3mmHg/L/phút

Bảng 2.1 Bảng phân loại TAP theo huyết động

2.2. Phân loại:

Phân loại lâm sàng tăng áp phổi dựa vào cơ chế sinh lý bệnh, biểu hiện lâm sàng, đặc điểm huyết động và cách điều trị. Phân loại này có mục đích hệ thống hóa chẩn đoán và điều trị nhóm bệnh phức tạp dẫn đến tăng áp phổi.

 Bảng 2.2 Phân loại lâm sàng tăng áp phổi

Hình 2.1 Các thể bệnh và điều trị tăng áp phổi

3. Chẩn đoán tăng áp phổi

3.1. Mục tiêu

• Phát hiện sớm và xác định chẩn đoán có tăng áp phổi
• Tìm nguyên nhân tăng áp phổi để điều trị theo nguyên nhân

3.2. Triệu chứng cơ năng

Bệnh nhân thường có biểu hiện lâm sàng của suy tim phải với các triệu chứng tăng dần theo mức độ nặng của bệnh.

Giai đoạn sớm:

• Khó thở khi gắng sức
• Mau mệt mỏi, kiệt sức
• Khó thở khi cúi người ra trước
• Hồi hộp
• Chướng bụng, buồn nôn
• Ho ra máu
• Ngất

Giai đoạn trễ khi động mạch phổi dãn lớn có thể gây các triệu chứng chèn ép cơ học:

• Khàn tiếng do thần kinh quặt ngược thanh quản trái bị chèn ép
• Đau ngực khi gắng sức do động mạch vành trái bị chèn ép
• Ho, khó thở, khò khè, nhiễm trùng đường hô hấp, xẹp phổi

3.3. Triệu chứng thực thể

• Người bệnh có các dấu hiệu lâm sàng của suy tim phải như phù chân, báng bụng, gan to, tĩnh mạch cổ nổi.
• Triệu chứng của bệnh tăng áp phổi như tím trung ương hoặc ngoại biên, độ bão hòa oxy máu giảm, nghe tim tiếng T2 mạnh, âm thổi tâm thu ở bờ trái xương ức do hở van ba lá, âm thổi tâm trương vùng đáy tim do hở van động mạch phổi.
• Ngoài ra, người có thể có các triệu chứng thực thể của bệnh nền gây tăng áp phổi như ran ở phổi, lồng ngực hình thùng, ngón tay dùi trống, dãn tĩnh mạch chi dưới,…

3.4. Điện tâm đồ

• Có thể thấy trục QRS lệch phải, block nhánh phải, lớn nhĩ phải, lớn thất phải, tăng gánh tâm thu thất phải trên điện tâm đồ 12 chuyển đạo. Ngoài ra, có thể có ngoại tâm thu nhĩ, nhịp nhanh nhĩ hoặc rung nhĩ.
• Điện tâm đồ bình thường không loại trừ được bệnh tăng áp phổi.

3.5. X-quang tim phổi:

• Các dấu hiệu bất thường trên X-quang ngực trong bệnh tăng áp phổi gồm dấu hiệu lớn nhĩ phải, lớn thất phải, cung động mạch phổi dãn. Ngoài ra, có thể thấy dấu hiệu bất thường của bệnh nền gây tăng áp phổi như bệnh phổi mạn tính, bệnh tim trái với bóng tim to,…
• Tuy nhiên, X-quang phổi bình thường cũng không loại trừ được bệnh này.

3.6. Siêu âm tim

• Dấu hiệu của tăng áp động mạch phổi gồm dày thất phải, dãn nhĩ phải, dãn động mạch phổi, thất trái có dạng “D-shape”, vách liên thất phẳng hoặc bị đẩy lệch sang trái trong thời kỳ tâm trương, hở van 3 lá, hở van động mạch phổi, áp lực động mạch phổi tâm thu qua dòng hở van 3 lá tăng cao. Các dấu hiệu thường gặp trên siêu âm tim của bệnh tăng áp phổi được trình bày ở Bảng 3.1

Bảng 3.1 Các dấu hiệu siêu âm tim và mặt cắt tương ứng của bệnh tăng áp phổi

• Dấu hiệu của bệnh tim nền như tim bẩm sinh có luồng thông ở tầng nhĩ, tầng thất hay đại động mạch, bệnh van tim trái, bệnh cơ tim,…
• Trong nhóm bệnh tim bẩm sinh, một số trường hợp cần làm thêm siêu âm tim qua thực quản, chụp CT tim hoặc MRI tim như thông liên nhĩ thể xoang vành, thông liên nhĩ xoang tĩnh mạch, hồi lưu bất thường tĩnh mạch phổi,…

Bảng 3.2 Các dấu hiệu siêu âm tim gợi ý bệnh tăng áp phổi

3.7. Khí máu động mạch – Test về chức năng phổi

• Các test về chức năng phổi gồm phế dung ký, đo dung tích phổi, đo khả năng khuếch tán CO của phổi (DLCO) và phân tích khí máu động mạch giúp phân biệt các nhóm bệnh tăng áp phổi, bệnh đi kèm, chỉ định điều trị oxy liệu pháp và độ nặng của bệnh.
• Trong tăng áp động mạch phổi, PaO2 bình thường hoặc giảm nhẹ. Trường hợp PaO2 giảm nặng gặp trong tồn tại lỗ bầu dục, thông liên nhĩ luồng thông phải – trái, bệnh gan hoặc các bệnh lý liên quan đến DLCO thấp. PaCO2 thường thấp hơn bình thường do tình trạng tăng thông khí, có liên quan đến dự hậu không tốt; rất ít khi PaCO2 tăng do tình trạng giảm thông khí phế nang.

3.8. Xạ ký tưới máu phổi:

Chỉ định ở bệnh nhân mới phát hiện tăng áp phổi để loại trừ hay tìm các dấu hiệu của bệnh tăng áp phổi do huyết khối thuyên tắc mạn tính (Chronic Thromboembolic Pulmonary Hypertension, CTEPH). Nếu không có bệnh nhu mô phổi, xạ ký tưới máu phổi bình thường thì có khả năng loại trừ CTEPH đến 98%.

3.9. Chụp CT ngực cản quang

Chụp CT ngực được chỉ định ở bệnh nhân khó thở không rõ nguyên nhân, nghi do tăng áp phổi. Các dấu hiệu trên CT gợi ý tăng áp phổi gồm động mạch phổi dãn lớn, tỉ lệ ĐMP/ĐMC > 0.9, dãn lớn buồng tim phải. Khi có 3 thông số sau: [1] ĐMP ≥ 30 mm, [2] bề dày RVOT ≥ 6 mm, [3] tỉ lệ thất phải/thất trái ≥1 thì khả năng cao có tăng áp phổi.

Chụp động mạch phổi có cản quang giúp phát hiện tăng áp phổi do huyết khối thuyên tắc mạn tính (CTEPH) qua các dấu hiệu trực tiếp và gián tiếp như khiếm khuyết đổ đầy trong động mạch phổi, động mạch phổi co nhỏ/dãn lớn, tưới máu phổi dạng khảm, dãn lớn động mạch phế quản. Phương pháp này chẩn đoán CTEPH có độ nhạy cảm 76%, độ đặc hiệu 96%. Chụp CT ngực cản quang còn giúp phát hiện bất thường bệnh tim mạch như bệnh tim bẩm sinh có luồng thông trong tim, hồi lưu bất thường tĩnh mạch phổi, còn tồn tại ống động mạch, dị dạng động tĩnh mạch phổi.

3.10.   Cộng hưởng từ tim

Chỉ định để đánh giá hình dạng, kích thước và chức năng tim phải. Chụp cộng hưởng từ tim có thuốc tương phản giúp đo được dòng máu trong động mạch phổi, động mạch chủ và tĩnh mạch chủ dưới, tính được thể tích nhát bóp, luồng thông trong tim và dòng trào ngược. Phương pháp này có thể giúp phát hiện sớm các dấu hiệu gợi ý tăng áp phổi và bệnh tim bẩm sinh, tuy nhiên, không đo được áp lực động mạch phổi.

3.11. Xét nghiệm máu

Các xét nghiệm máu cần làm để tìm nguyên nhân và biến chứng tăng áp phổi, bệnh khác đi kèm như công thức máu, điện giải đồ, chức năng thận, chức năng gan, acid uric, sắt huyết thanh, chức năng tuyến giáp, NT-ProBNP hoặc BNP. Ngoài ra cần làm thêm huyết thanh chẩn đoán HIV và viêm gan siêu vi. Các xét nghiệm về bệnh tự miễn như kháng thể kháng nhân ANA, kháng thể kháng centromere, anti-Ro, tầm soát hội chứng kháng phospholipid ở bệnh nhân CTEPH (anticardiolipin, Lupus anticoagulant, anti beta-2 glycoprotein).

3.12. Siêu âm bụng tổng quát

Chỉ định siêu âm bụng tổng quát để đánh giá bệnh lý của gan, tăng áp lực tĩnh mạch cửa, thông nối tĩnh mạch cửa-tĩnh mạch chủ (dị dạng Abernethy).

3.13. Trắc nghiệm gắng sức tim phổi

Trắc nghiệm gắng sức tim phổi giúp đánh giá cơ chế sinh lý bệnh nền làm người bệnh giảm khả năng gắng sức. Bệnh nhân tăng áp động mạch phổi có kiểu bất thường điển hình sau: PETCO2 thấp, VE/VCO2 cao, VO2/ HR thấp và VO2 thấp. Những bất thường này nên nghĩ ngay đến bệnh mạch máu phổi. Ở những người có nguy cơ cao bị tăng áp động mạch phổi như bệnh xơ cứng hệ thống, nếu VO2 đỉnh bình thường có thể loại bỏ chẩn đoán tăng áp động mạch phổi.

3.14. Thông tim phải

Thông tim phải là tiêu chuẩn vàng để chẩn đoán và phân loại tăng áp phổi. Ngoài ra, thông tim phải còn được chỉ định trong bệnh tim bẩm sinh có luồng thông trong tim và đánh giá trước khi ghép tim và phổi.

Chống chỉ định thông tim phải khi biết có u hoặc huyết khối trong nhĩ phải hoặc thất phải, mới đặt máy tạo nhịp < 1 tháng, van 3 lá cơ học, kẹp van 3 lá (TriClip) và nhiễm trùng cấp.

Các thông số cần đo đạc trong khi làm thông tim được trình bày trong Bảng 3.3

Bảng 3.3 Các thông số huyết động trong thông tim phải

WU, đơn vị Wood. (a) mẫu máu lấy từ động mạch phổi; ngăn đo oxy để loại trừ luồng thông trong tim khi SvO2 > 75%. (b) PVR, (mPAP−PAWP)/CO. (c) TPR, mPAP/CO. (d) PAC, SV/(sPAP−dPAP).

3.15. Trắc nghiệm dãn mạch trong thông tim phải

Mục đích của trắc nghiệm này nếu có đáp ứng với thuốc dãn mạch thì người bệnh sẽ được điều trị với thuốc ức chế canxi liều cao. Nghiệm pháp này chỉ thực hiện ở bệnh nhân tăng áp động mạch phổi vô căn, tăng áp động mạch phổi di truyền và tăng áp động mạch phổi do thuốc hoặc độc chất.

Chất được sử dụng là nitrite oxid, iloprost hít hoặc epoprostenol truyền tĩnh mạch. Trắc nghiệm được gọi là có đáp ứng hay dương tính khi áp lực động mạch phổi trung bình giảm ≥ 10 mmHg, đạt giá trị tuyệt đối ≤ 40 mmHg với cung lượng tim tăng hoặc không thay đổi.

Bảng 3.4 Khuyến cáo thông tim phải và trắc nghiệm dãn mạch

(Vui lòng xem tiếp trong kỳ sau)

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