Before we are Born: Fetal Diagnosis of Congenital Heart Disease

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Before we are Born: Fetal Diagnosis of Congenital Heart Disease Mohamed Sulaiman, MD Pediatric cardiologist Kidsheart: American Fetal & Children's Heart Center Dubai Healthcare City, Dubai-UAE

First Pediatric Cardiology Symposium of Kidsheart

Disclosure No thing to disclose except: I am the Co-founder of Kidsheart : American Fetal and Children heart center.

Objectives: Prenatal detection of CHD Indication for fetal echo. Program for fetal echocardiography in UAE.

Prenatal Ultrasound 1979 of academic interest only. It should not be used to influence clinical judgment. 2013: diagnosis of CCVM as early as 12 weeks of gestation assess physiology of heart failure diagnosis of cardiac arrhythmias and guide treatment gain insight into the natural history of congenial and acquired cardiac defects

Why screen for CHD CHD are the most common lethal congenital malformations and The most common congenital defect (8/1000 live births) The majority of CHD are from low risk pregnancies

An Ultrasound is an Ultrasound? Level I: Level II: Fetal size and Gestational age cardiac motion OB/Technician ( do and read) Fetal Structural Survey four chamber view Perinatologist/Radiologist Level III: Fetal Echocardiogram ( Pediatric Cardiologist/some prenatologist)

Four Chamber View (Level II)

Detection of CHD : How Good is a Screening Ultrasound? Accurate 4 chamber view detects 4 50 % of serious CHD. DOES NOT detect CHD with outflow tract anomalies that can be ductal dependent lesions

Abnormalities Detectable by 4 Chamber Screening Examination Single Ventricle Compete AV septal defect

Cardiac Surgery on Single Ventricles at UOC (July 1999 April 2008) Diagnosed before birth 28% SV 11% HLH 37% HRJ 18%

Hypoplastic left heart Syndrom

Cardiac Surgery on Complete AV Septal Defect at UOC (July 1999 April 2008) Diagnosed before birth 8%

Tricuspid valve dysplasia Tricuspid atresia

Detecting Fetal CHD: Non-Selected Population at 18-24 Weeks 5347 fetuses evaluated with 4 chamber view PLUS follow-up scan if imaging suboptimal in first scan Scan time (US and 4-C heart) 30 minutes Detected 48% of CHD Incorporating great vessel views increased dectection to 78% Achiron,R. Br Med J 304; 671-4 (1992)

Abnormalities Detectable by 4 Chamber Plus Outflow Tract Exams Transposition of the great vessels Tetralogy of Fallot Persistent truncus arteriosus Interrupted aortic arch

Improving the detection rate Adding the LVOT to the scan Adding the RVOT to the scan

Transposition of Great Arteies

TOF DORV

Rhabdomyoma

Pulsed Doppler

Color Flow Mapping

Detecting Fetal CHD: Unpublished data from Tawam hospital database Detection rate is 92 %. Initially was 87 %, then improved to 96 % in the last few years

Prenatal Detection of CHD 1. CHD almost never manifest before birth except valve regurgitation 2. Screening ultrasound for all patients at 20 22 weeks. 3. We can not screen every one with fetal echo ( time and money) 4. Screen high risk, and suspicious screening ultrasound.

Risk factors for CHD Maternal Risk 1. Diabetes 2. Anti-SSA/SSB (Ro/La) antibodies 3. Family hx CHD or cardiac syndrome 4. Teratogen exposure 5. Abnormal serum screening

Family History of CHD If either parent: risk is 2.5 4% If sibling: risk is 1 4 % If more than one sibling affected risk is 3 10% Highest recurrence risk is for left outflow obstruction (AS, COA, HLH variants) : 10 15%

Family History of Syndromes with Cardiac Involvement Williams syndrome Marfan syndrome Tuberous sclerosis 22 q 11 Deletion syndrome Hypertrophic cardiomyopathy Noonan syndrome

Fetal Risk Risk factors for CHD 1. Non-cardiac anomaly 2. Suspected cardiac defect ( Very important) 3. Chromosome abnormalities. 4. Increased nuchal translucency 5. Hydrops fetalis 6. Arrhythmia

Chromosome Abnormalities and CHD Trisomy 21: 40% CAVCD, VSD, TOF Trisomy 18: 90% VSD, DORV, Polyvalver disease Trisomy 13: 80% VSD

Miscellaneous Hydrops or isolated effusion Suspected twin: twin transfusion IUGR IVF

Abnormal Rate or Rhythm Bradycardia HR < 100 bpm Tacycardia HR > 180 bpm Irregular rhythm

M Mode and arrhythmia

Advantages of Prenatal Diagnosis Parental reassurance Multidisciplinary counseling Therapeutic intervention (arrhythmias not structural defects) IMPROVED OUTCOME No surprises after birth

Pediatric Fetal Cardiology program Multidisciplinary team: Prenatal OBGYN Pediatric Cardiologist Pediatric Fetal Cardiology program NICU team

Why we are doing all of this?

Thank you

Where is this Park located?