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pulmonary hypertension crisis in Patient with Tetralogy of Fallot and Mixed Total Anomalous pulmonary Vein Connection after the Primary Correction:A Rare Case Report

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Congenital Heart Disease 2023年第6期18卷 671-678页

作者： Dian Kesumarini Yunita Widyastuti Cindy Elfira Boom Lucia Kris Dinarti Department of Anesthesia and Intensive Therapy National Cardiovascular Center Harapan KitaJakartaIndonesia Doctoral Program Faculty of Medicine and Public HealthUniversity of Gadjah MadaYogyakartaIndonesia Department of Anesthesia and Intensive Therapy Universitas Gadjah Mada/Dr.Sardjito HospitalYogyakartaIndonesia Department of Cardiology and Vascular Medicine Universitas Gadjah Mada/Dr.Sardjito HospitalYogyakartaIndonesia

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.

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

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