Perinatal Management Of Complex CHD
Perinatal Management of Complex CHDEverything you aorta know: HLHS/TGA/HeterotaxyYalda Afshar, MD, PhDDivision of Maternal Fetal MedicineUniversity of California, Los AngelesOctober 16, 2021
Outline: following the flow1.2.3.4.5.6.7.Artist: Leanne PearceIt takes a teamNormalize pregnancyLooking for extra cardiac anomaliesGenetic testingCounselingAntenatal testingDelivery planning
Dedication and InspirationHappynd2birthday to Theo and Charlie
Multi-disciplinary approach to fetal CHDOB / Maternal Fetal Medicine Screening for fetal CHD Looking for extra cardiacanomalies Genetic testingNursing CounselingSocial Work Antenatal testingGenetics Delivery planningNICUCT surgeryPediatric Cardiology Confirmation of CHD Counseling Outcome, Surgery, QoLY Postnatal treatmentrecommendations Postnatal management
Pregnancy is not a diseaseDid I dosomething thatcaused this?Do I need acesarean?Is baby saferoutside?
Extracardiac anomaliesOB / Maternal Fetal Medicine Screening for fetal CHD Looking for extra cardiac anomalies Genetic testing Counseling Antenatal testing Delivery planning
Extracardiac abnormalities Incidence of CHD in presence of 1 extracardiacmalformations is 20-45%, depending on the population,malformation type, and gestational age of ultrasound Cardiac malformations have been observed in 30% of omphaloceles,20% of duodenal atresia,30% of CDHs,5-15% of CNS malformations,Up to 71% of GU abnormalities.Donofrio, MT. Circulation, 2014
Extracardiac abnormalitiesDonofrio, MT. Circulation, 2014
Genetic Evolution of the Fetus Congenital abnormalities occur in 3% of newborns, 5% risk forgenetically based disorder by age 5 y/o. Many of these abnormalities have underlying genetic etiology. Determine a precise diagnosis because Important to understand the underlying etiology Important to understand the prognosis Important to know the recurrence risk
Genetic TestingOB / Maternal Fetal Medicine Screening for fetal CHD Looking for extra cardiac anomalies Genetic testing Counseling Antenatal testing Delivery planning
Onset of Genetic Syndromes: Focus on FetalFETAL Identifying women at increased risk for T21 been the focus of prenatal screening programs. Programs for universal parental carrier screening for AR disorders and ethnicity-based carrierscreening to identify parents at risk of having an affected child.
Invasive Diagnostic Testing Began in 1966 with cultured cells from amniotic fluid Goal inform parents with information about the risk of birth defects orgenetic disorders Provide rational information about how to manage risk associated withcongenital anomalies / genetic disorder Options: PGT, CVS, amniocentesis
Chromosomal microarrays are asubstantial leap forward CMA detect CNVs (a change in amount of DNA) and majorchromosomal aneuploidy Standard of care – NEJM, Wapner, et al. 2012 Prospective study n 4,406, CMA was successful in 98.8% of cases(aneuploidy, unbalanced translocation) structural anomalies 6% hadclinically relevant deletion/duplication, AMA or screening – 1.6%Chromosomal Del/Dup Syndromescredit: Deb KrakowWeise, et al, 2012
Look Back at the History of cfDNA-based NIPT:14 Years – from Discovery to Commercial Products200819972011Dennis Lo, PhDHong Kong Chinese University
NIPT/cfDNA: challenges Distinguish fetal DNA False positives ContaminationUnrecognized / vanishing twinPlacental mosaicismLow-level maternal mosaicism False negatives Failure to extract adequate material Individual variation in the amount of cfDNA
Determining a Prenatal DIAGNOSIS Important to understand the underlying etiology Important to understand the prognosis Important to know the recurrence risk
Risk of aneuploidy by cardiac malformationDonofrio, MT. Circulation, 2014
Prenatal CounselingOB / Maternal Fetal Medicine Screening for fetal CHD Looking for extra cardiac anomalies Genetic testing Counseling Antenatal Testing Delivery planning
Prenatal counseling requires a unified frontOBFamilyPlanningMFMNICUSurgeryPrenatalCare TeamGeneticsSWCardsReview the Literature,Discuss, Debate, SharedDecision Making
Multi-disciplinary approach to fetal CHDOB / Maternal Fetal Medicine Screening for fetal CHD Looking for extra cardiac anomalies Genetic testing Counseling Antenatal testing Delivery planning
Antenatal testing
Antenatal testingACOG PB #194, 2016UCfC Data, Afshar Y, et al, JAHA, 2021
Feto-placental circulation and cardiac output:rationale for serial growth scans
More than meets the heart:CHD and brain development 41 term w/ CHD 29 TGA 12 single ventricleN Engl J Med 2007; 357:1928-1938 Control (n 55) vs CHD (n 50)
Multi-disciplinary approach to fetal CHDOB / Maternal Fetal Medicine Screening for fetal CHD Looking for extra cardiac anomalies Genetic testing Counseling Antenatal testing Delivery planning
Delivery planning A prenatal diagnosis of CHD has been associated with lowerbirth weights and earlier gestational age of delivery Both have been linked to decreased survival andneurodevelopmental outcomesKipps Am J Cardiol 2011, Levey Pediatr Cardiol 2010Costello Circulation 2014, Goff J Thorac Cardiovasc Surg 2012
Delivery planning recommendations Recommend routine delivery/induction at ³ 39 0/7 wks Earlier if required for any other medically-indicated obstetrical reason Recommend vaginal delivery with cesarean delivery only forobstetrical indications Consider cesarean delivery for a fetus with complete heart block or ifdelivery coordination is needed for CHD category 4 lesions Recommend prenatal genetic counseling
Level of Care Assignment and Coordination Action Plan
Decreasing cesarean and early-term deliveryStandardized clinical assessment and management pathway (SCAMP) for CHD decreased early-term birth and cesarean birth
Afshar Y, et al. JAHA, 2021
Today, more adults (than children) are living with CHDEuropean Heart Journal (2011) 32, 3147–3197doi:10.1093/eurheartj/ehr218ESC GUIDELINESESC Guidelines on the management ofcardiovascular diseases during pregnancyThe Task Force on the Management of Cardiovascular Diseasesduring Pregnancy of the European Society of Cardiology (ESC)Congenital Heart Disease inAuthors/Task Force Members: Vera Regitz-Zagrosek (Chairperson) (Germany) ,PregnancyCarina Blomstrom Lundqvist (Sweden), Claudio Borghi (Italy), Renata Cifkova*(Czech Republic), Rafael Ferreira (Portugal), Jean-Michel Foidart† (Belgium),a,*, ManishaWayneJ. Franklin,Gandhi, MDb (Germany), Bulent GorenekJ. SimonR. GibbsMD(UK),Christa Gohlke-Baerwolf(Turkey), Bernard Iung (France), Mike Kirby (UK), Angela H.E.M. Maas(The Netherlands), Joao Morais (Portugal), Petros Nihoyannopoulos (UK),KEYWORDSG. Pieper (The Netherlands), Patrizia Presbitero (Italy),!PetronellaPregnancy ! Congenital heart disease ! CARPREG score ! ContraindicationsJolienW.Roos-Hesselink(The Netherlands),Maria Schaufelberger! Infective endocarditis ! Eisenmengersyndrome ! Pulmonaryhypertension ! (Sweden),Tetralogy of FallotUte Seeland (Germany), Lucia Torracca (Italy).AHA SCIENTIFIC STATEMENTDownloaded from http://eurheartj.oxfordjournals.org/ by guest on November 29, 2016Endorsed by the European Society of Gynecology (ESG), the Association forEuropean Paediatric Cardiology (AEPC), and the German Society for GenderMedicine (DGesGM)Management of Pregnancy in Patients WithComplex Congenital Heart DiseaseESC Committee for Practice Guidelines (CPG): Jeroen Bax (CPG Chairperson) (The Netherlands),KEYPOINTSAngeloAuricchio (Switzerland), Helmut Baumgartner (Germany), Claudio Ceconi (Italy), Veronica Dean (France),Christi Deaton (UK), Robert Fagard (Belgium), Christian Funck-Brentano (France), David Hasdai (Israel),!ArnoFor uhani Knuuti(Finland), Philippe(Belgium),isTheresaMcDonagh(UK),!CyrilIt is Moulinimportantfor theto understandthe (The Netherlands),BogdanhemodynamicA. Popescu Germany),Sirnes (Norway),AdamTorbicki(Poland),Alec Vahanian(France),!UdoPregnantpatientscanPerbe Antonrisk-stratifiedinto low,medium,andhigh cardiacrisk basedon the CARStephan Windecker (Switzerland).PREG Risk Score.! Cardiac absolute contraindications against pregnancy include: pulmonary hypertension, Marfansyndrome with dilated aortic root (4 cm), severe left heart obstruction, and systemic ventricularfunction less than 30%.A Scientific Statement for Healthcare Professionals From the AmericanHeart Association
Discussing a Reproductive Life Plan Do you plan to have any (more) children at any time in your future?– If YES: How many? When? Family planning method until you are ready?– If NO: Family planning method to avoid pregnancy? How sure are you that can use without problems? People’s plans change . . . reversible?
Questionsyafshar@mednet.ucla.edu@yafshar
Medicine, Baylor College of Medicine, 6621 Fannin Street, 20th Floor West Tower, MC 19-345C, Houston, TX 77030, USA; b Departments of Obstetrics and Gynecology, Baylor College of Medicine, Houston, TX 77030, USA
Hypoplastic left heart syndrome 6% D-Transposition 5%. CHD in Adults 30,000 babies born with CHD per year 20,000 surgeries for CHD per year 85% survive into adulthood . Rarely: SVC, IVC, right atrium, or left subclavian vein “Very loud murmur” heard prior to hospital discharge
Perinatal anxiety or perinatal OCD – some people develop anxiety problems during pregnancy or in the first year after giving birth. See our resource on perinatal anxiety and perinatal OCD for more information. "It's like a swarm of bees just buzzing buzzing buzzing, not
Comprehensive information about the psychosocial assessment, treatment and care of all women is available from beyondblue s (2011) Perinatal Clinical Practice Guidelines. . managing perinatal mental health disorders in primary care For women and their families Understanding perinatal depression and anxiety yer
The organisation and provision of perinatal mental health services may vary greatly from country to country [27]. In this paper, we aimed to compare the perinatal mental health services provided in the UK and in Switzerland. The UK has a longstanding tradition in providing perinatal-psychi-atric services, and there is also a tradition of continuous
Treatment for perinatal depression is important for the health of both the mother and the baby, as perinatal depression can have serious health effects on both. With proper treatment, most women feel better and their symptoms improve. Treatment for perinatal depression often includes therapy, medications, or a . combination of the two.
Bereavement Doula RTS Perinatal and Infant Loss RTS Coordinator Training Trauma Informed Care Ordained Minister, PC(USA) Bereaved Parent Learning Objectives Summarize grief related needs of parents experiencing perinatal or infant loss. Identify three ways to verbally support parents experiencing perinatal or infant loss that will
disease. This also can lead to PH, categorized as group 3 disease. Finally, patients with CHD may develop thromboembolic disease, which can lead to group 4 disease and/or can have concom-itant systemic disease that leads to group 5 dis-ease. The most complex forms of PAH-CHD have been classified as group 5. These include
Connect module An exceptional labor and delivery solution used in the delivery of over 40 million babies.1 Plus an interconnected view of your preferred enterprise EMR or other system. Centricity Perinatal – Connect enables you to see critical perinatal information in context with other clinical data—
