Pulmonary Hypertension Crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous Pulmonary Vein Connection after the Primary Correction:A Rare Case Report

作     者：Dian Kesumarini Yunita Widyastuti Cindy Elfira Boom Lucia Kris Dinarti 

作者机构：Department of Anesthesia and Intensive TherapyNational Cardiovascular Center Harapan KitaJakartaIndonesia Doctoral ProgramFaculty of Medicine and Public HealthUniversity of Gadjah MadaYogyakartaIndonesia Department of Anesthesia and Intensive TherapyUniversitas Gadjah Mada/Dr.Sardjito HospitalYogyakartaIndonesia Department of Cardiology and Vascular MedicineUniversitas Gadjah Mada/Dr.Sardjito HospitalYogyakartaIndonesia 

出 版 物：《Congenital Heart Disease》 (先天性心脏病（英文）)

年 卷 期：2023年第18卷第6期

页      面：671-678页

核心收录：

学科分类：1002[医学-临床医学] 100201[医学-内科学(含：心血管病、血液病、呼吸系病、消化系病、内分泌与代谢病、肾病、风湿病、传染病)] 10[医学] 

基　　金：The report was conducted in accordance with the Nuremberg Code and Declaration of Helsinki and the protocol was approved by the Institutional Review Board of National Cardiovascular Center Harapan Kita(No.LB.02.01/VII/037/KEP037/2022) 

主　　题：Pulmonary hypertension crisis major aortopulmonary collateral arteries tetralogy of Fallot total anomalous pulmonary vein connection congenital heart disease case report 

摘      要：Tetralogy of Fallot(TOF)with total anomalous pulmonary vein connections(TAPVC)is a rare type of complex congenital heart disease among all TOF ***-presentation of major aortopulmonary collateral arteries(MAPCAs)compensates for the lack of central pulmonary bloodflow and decreases the severity of right-to-left shunting in *** present a case of a 2-year-old child with complex diagnoses of TOF,TAPVC,a large secun-dum atrial septal defect(ASD),and intraoperatively identified *** underwent surgery to repair the TAPVC,valve-sparing reconstruction of the right ventricular outflow tract,interventricular defect closure,and the creation of patent foramen ovale(PFO).After the operation,hemodynamic instability happened along with sudden blood pressure drop,desaturation,and increased central venous pressure,which subsided after adminis-tering inhalational nitric oxide(NO).A postoperative pulmonary hypertension crisis was suggested when the patient experienced recurrent symptoms after the termination of ***findings of a D-shaped left ventricle(LV),right-to-left PFO shunt and high tricuspid valve gradientfirmly established the *** was subsequently managed with continuous NO inhalation and sildenafil,which rendered a satisfactory *** TOF and TAPVC could be at particular risk of developing pulmonary hypertension crisis,especially in the presence of MAPCAs due to possible remodeling of the pulmonary ***,a relatively non-compliant LV function and small left atrial size may exacerbate the risk of developing postcapillary pulmonary hypertension after TAPVC repair.A successful postoperative outcome calls for a meticulous preoperative analysis of the anatomical lesions,as well as careful monitoring.