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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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The Potential of Circular RNAs as Biomarkers in Pulmonary Arterial Hypertension Related to Congenital Heart Disease

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Congenital Heart Disease 2024年第4期19卷 375-388页

作者： Fajri Marindra Siregar Sofia Mubarika Haryana Dyah Wulan Anggrahini lucia kris dinarti Anggoro Budi Hartopo Doctorate Program of Medical and Health Science Faculty of MedicinePublic Health and NursingUniversitas Gadjah MadaYogyakarta55281Indonesia Department of Biochemistry Faculty of MedicineUniversitas RiauPekanbaru28133Indonesia Department of Histology and Cell Biology Faculty of MedicinePublic Health and NursingUniversitas Gadjah MadaYogyakarta55281Indonesia Department of Cardiology and Vascular Medicine Faculty of MedicinePublic Health and NursingUniversitas Gadjah Mada Dr.Sardjito HospitalYogyakarta55281Indonesia

A particular type of endogenous noncoding RNAs known as circular RNAs(circRNAs)has now become possible biomarkers for several diseases because of their stability and tissue-speciﬁc expression *** might play a role in various of biological *** identiﬁcation of particular circRNAs dysregulated in pulmonary arterial hypertension(PAH)raises the possibility of these molecules serving as biomarkers for the disease’s early diagnosis and *** review mainly summarizes the role and potential of circRNA as a future biomarker in PAH related to congenital heart *** study presented several potential circRNA targets as diagnostic biomarkers for PAH,discussed their biological functions,and addressed the challenges that need to be considered for their application in clinical settings.

关键词： Biomarker circular RNA congenital heart disease pulmonary arterial hypertension

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Reliability of Echocardiographic Pulmonary Vascular Resistance to Screen for the New Definition of Precapillary Pulmonary Hypertension in Uncorrected Secundum Atrial Septal Defect

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Congenital Heart Disease 2024年第3期19卷 315-324页

作者： Risalina Myrtha Hasanah Mumpuni Real Kusumanjaya Marsam Dyah Wulan Anggrahini Anggoro Budi Hartopo lucia kris dinarti Department of Cardiology and Vascular Medicine Faculty of MedicineUniversitas Sebelas MaretSurakarta57126Indonesia Department of Cardiology and Vascular Medicine Universitas Sebelas Maret HospitalSukoharjo57161Indonesia Department of Cardiology and Vascular Medicine Faculty of MedicinePublic Healthand NursingUniversitas Gadjah MadaYogyakarta55281Indonesia Department of Cardiology and Vascular Medicine Dr.Sardjito HospitalYogyakarta55284Indonesia

Background and Objective:The most feared complication of uncorrected secundum Atrial Septal Defect(ASD)is pulmonary arterial hypertension(PAH).Pulmonary vascular resistance(PVR)is crucial in detecting precapil-lary pulmonary hypertension(PH)to guide the need for PAH-specific *** is a change in the cut-off value of PVR according to the recently updated PH *** echocardiographic PVR(PVRecho)correlates to PVR by right heart catheterization(RHC)(PVRcath)according to the new guidelines has not been *** aim of this study is to determine the reliability of PVRecho in detecting PAH in Uncorrected Ostium Secundum ASD based on the current updated guideline and to help screen the high PVR ***:429 ostium secun-dum ASD in the COngenital HeARt Disease in Adult and Pulmonary Hypertension(COHARD-PH)registry was divided into three groups according to the *** was calculated using Abbas’Formula and compared the its gold standard,the *** correlation between the two methods was *** Bland-Altman plot was used to analyze the agreement between the two *** operating characteristics(ROC)analysis was used to determine the PVRecho cut-off value for high ***:The majority of the population(63.5%)had high *** gender dominated the study population(84%).PVR_(echo) was significantly correlated with PVRcath(r=0.6225,p1.62 WU showed good dis-criminatory power in determining high PVR.

关键词： Pulmonary vascular resistance atrial septal defect echocardiography

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