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KHÔNG LỖ VAN LÁ 1- 3% bệnh TBS - 1/10000 - 20000 sơ sinh Tam sắc thể 21 - Hội chứng “Mắt mèo” BS Đào Hữu Trung ĐỊNH NGHĨA - ĐỊNH DANH PHÂN LOẠI - Tim thất - Tâm thất Trái - Khơng nối tiếp NT - phải - Situs Solitus - Phân loại : I Ia Ib Ic Khơng có CV ĐĐM II IIa IIb IIc CV ĐĐM Type D III CV ĐĐM Type L Giải phẫu bệnh lý  Van - Khơng phát triển - Xơ hố - Màng  Nhĩ phải - Dày - 10% juxtaposition atriale TLN nhiều týp  Thất trái - Buồng thất - Dày dãn tùy thuộc lượng máu lên phổi  Thất phải - Thất phụ thành phần  Thông liên thất thường hạn chế Sinh lý bệnh học  yếu tố - Kích thước lỗ TLN - Có hay khơng hẹp ĐMP + Ch/vị ? - Kích thước lỗ thơng TLT  Thơng thường - Tăng lượng máu lên phổi - Hiếm - Giảm lượng máu lên phổi - Nhiều Anatomic classification of tricuspid atresia In about 70% of cases the great arteries are normally related, and there is a small ventricular septal defect (VDS) with associated hypoplasia of the pulmonary artery (PA) When the great arteries are transposed, the VSD is usually large, and the Pas are large with increased pulmonary blood flow AS, aortic stenosis ; D-TGA, complete TGA ; L-TGA, congenitally corrected TGA ; PA, pulmonary atresia ; PS, pulmonary stenosis ; Sub AS, subaortic stenosis ; Sub PS, subpulmonary stenosis ; TGA, transposition of the great arteries ; VSD, ventricular septal defect (Data from Keith JD, Rowe RD, Vlad P : Heart Disease in Infancy and Childhood, 3rd ed New York, Macmillan, 1978) Classification by associated lesions Type I, normally related great arteries ; II, D-transposed great arteries : a pulmonary atresia; b subpulmonary or pulmonary valvular stenosis (reduced pulmonary blood flow); c no pulmonary stenosis (normal or increased pulmonary blood flow), and III, L-transposed great arteries (Adopted from Edwards JE, Burchell HB; congenital tricuspid atresia : A classification Med Clin North Am 33 : 1177, 1949) Factors that determine physiology : VSD : if tiny, then the RV is severely hypoplastic and VR –related great artery receives no direct flow If large, then the RV can even be of normal size Stenosis of semilunar valve arising from the RV ; this determines the volume of flow into its great artery Great vessel relation : TGA or normally related Patient to left has : Tricuspid atresia + small/moderate VSD ; PS ; normally related great arteries Restrictive VSD makes RV small ; small RV and PS make MPA small ; Two above factors make the Qp inadequate Patient to left has : Tricuspid atresia + normally related great arteries There is no VSD and therefore no RV ; without trans-RV flow in utero there is pulmonary atresia Pulmonary blood flow is ductal-dependent ; therefore, the patient needs a shunt Patient to left has : Tricuspid atresia + a small/moderate VSD ; AORTIC stenosis and TGA (Ao from RV and MPA from LV) The small VSD has reduced flow in utero, causing hypoplasia of the RV, aortic valve, and Aao FUNCTIONALLY, this child has hypoplastic left heart syndrome LÂM SÀNG thể phổ biến - Khơng CVĐĐM Tùy thuộc hẹp ĐMP Tím sớm - Có CVĐĐM máu lên phổi Thường kèm tăng lưu lượng Suy tim sớm CẬN LÂM SÀNG ° ECG Trục lệch trái - Phì NP - Phì TT (SV1V2 > RV5,V6) Sóng P thay đổi 80% ca D1, D2, aVF, V1 ° Xq TP - Dạng hẹp ĐMP: Tim khơng lớn Hình trứng Tuần hồn phổi giảm - Dạng khơng có hẹp ĐMP: Tim lớn Tuần hồn phổi tăng 10 24 Tiêu chuẩn (cổ điển) để phẫu thuật Fontan tối ưu (10 “điều răn” Fontan) Tuổi 4-15 Nhịp xoang Hệ thống tĩnh mạch : bình thường Thể tích nhĩ phải : bình thường Aùp lực ĐMP trung bình < 15mmHg Kháng lực ĐMP < đv Woods/m2 Tỉ lệ đường kính ĐMP/ĐMC > 0,75 Phân suất tống máu tâm thu > 0,60 Van hai lá, kín 10 ĐMP không bị vặn vẹo TL : Choussat 1980 25 Glenn ABCDPA can be constructed as a bidirectional Glenn shunt in which the superior vena cava is anastomosed to the confluent pulmonary arteries (From Castaneda AR, Jonas RA, Mayer JE, et al : Single ventricle with tricuspid atresia In Cardiac Surgery of the Neonate and Infant Philadelphia, WB Saunders, 1994, p.262 ; with permission) 26 Hemi Fontan 27 Original Fontan 28 Atrio-pulm Derivation atrio-pulmonaire Le toit de l’oreillette droite (OD ) a été anastomosé la face inférieure de l’artère pulmonaire droite (APD) proximale Tout le sang cave est ainsi dérivé l’arbre artériel pulmonaire car la communication inter-auriculaire (CIA) a été fermée 29 Superior caval reconstruction The superior cava is transected and the cardiac and cephalad ends are anastomosed to the pulmonary arterial confluence A septation patch placed within the right atrium completes cavocaval continuity and separates systemic from pulmonary venous drainage 30 Dérivation cavo-pulmonaires partielles Il n’y a qu’une seule veine cave supériure (VCS) située droite et recevant un tronc veineux innominé (TVI) La grande veine azygos (Az) a été sctionnée-suturée, la VCS a été sectionnée et son bout périphérique anastromosé l’artère pulmonaire droite (APD), ce qui permet une irrigation bidirectonnelle (flèches) du sang cave Il y a deux veines caves supérieures, droite (VCSD) et gauche (VCSG) On procède de la même facon qu’en A mais de facon bilatérale sur les artères pulmonaires droite (APD) et gauche (APG) 31 Exemples de circulation mixte Un montage cavo-pulmonaire supérieur bi-directonnel a été confectionné comme sur la figure 2A On peut y ajouter : une anas tomose systémico-pulmonaire gauche (BG), droite (BD) ou laisser libre la voie anérograde venctriculo-pulmonaire (VA) 32 Dérivation cavo-pulmonaire sub-totale C’est un montage identique celui de la Figure 2A Le grainage azygos (Az) la veine cave spérieure (VCS) apporte aux artères pulmonaires tout le sang cave inférieur mais ni le sang veineux coronaire qui reste drainé l’oreillette droite (OD) par le sinus coronaire (Sco), ni le sang des veines sus-hépatiques (VSH) qui débouchement directement dans l’OD 33 Principaux types de dérivation cavo-pulmonaire totale On a commencé par faire un montage cavo-pulmonaire supérieur bi-directionnel A : La CIA a été fermée et le bout cardiaque de la VCS anastomosé l’APD Le sinus coronaire se draine l’OD B : L’OD a été divisée par une pièce de tissu synthétique (en rouge) et le bout cardiaque de la VSC anastomosé l’APD Le sinus coronaire se draine maintenant l’oreillette gauche Une fenêtre peut être ou ne pas être percée dans la paroi du tunnel intra-auriculaire pour autoriser un shunt droitegauche (flèche rouge) C : La veine cave inférieure (VCI) a été anastomosée l’APD par un conduit prothétique extracardique (rouge) le long de la paroi externe de l’OD On peut aussi le fenestrer l’oreillete droite devenue “gauche” 34 Modified Fontan A variant of the Fontan operation in a patient with tricuspid atresia and associated transposition of the great arteries (aorta cut away to show details of operation) A : The main pulmonary aetery is divided, and the opening is extended into the right pulmonary artery behind the superior vena cava The superior vena cava is divided, and th e incision is extended into the base of the right atrial appendage B : The proximal stump of the main pulmonary artery has been over-sewn, the ustream end of the superior vena cava has been anastomosed to an opening in the right pulmonary artery, and the atrial septal defect has been closed with a patch C : The large opening in the pulmonary artery has been sewn tho the large opening in the superior vena cava and right atrium Now, both the inferior and superior venae cavae and no communications remain between the right and left sides of the heart 35 36 Currently pupular modified Fontan operation A Bidirectional Glenn operation or superior vena cava (SVC)-to-right pulmonary artery anastomosis B Cavocaval baffle-to-pulmonary artery (PA) connection, with or without fenestration See text for description of these procedures AO, aorta ; IVC, inferior vena cava ; LV, left ventricle ; RA, right atrium ; RV, right ventricle 37 The Damus-Stansel-Kaye uses the unobstructed pulmonary outflow tract to provide relief of systemic outflow obstruction The main pulmonary artery is anastomosed to the aorta Pulmonary blood flow is provided with a systemic to pulmonary artery shunt or a BCDPA TL : Laks H, Gates RN, Elami A, et al : Damus-Stansel-Kaye procedure : Technical modifications Ann Thorac Surg 54 : 169-172, 1992 ; with permission 38 ... A classification Med Clin North Am 33 : 1177, 1949) Factors that determine physiology : VSD : if tiny, then the RV is severely hypoplastic and VR –related great artery receives no direct flow... ventricular septal defect (Data from Keith JD, Rowe RD, Vlad P : Heart Disease in Infancy and Childhood, 3rd ed New York, Macmillan, 1978) Classification by associated lesions Type I, normally related... ventricular enlargement ; alarge notched P-wave may be present, indicating atrial enlargement (P-tricuspidale) 14 Mặt cắt buồng từ mỏm : van màng dầy, có TLT lớn kèm (A) Mặt cắt buồng sườn (B-C) : van