Fetal echocardiography provides several advantages for diagnosing and managing congenital heart disease: 1) It allows for counseling of parents on prognosis and treatment options, including the possibility of termination of pregnancy for major defects or chromosomal anomalies. 2) In some cases, it enables life-saving procedures to be performed on the mother or fetus before or during pregnancy. 3) It facilitates delivery planning at a specialized medical center near pediatric cardiac surgery.

1. IMPATTO
DELL’ECOCARDIOGRAFIA
FETALE SULL’OUTCOME
CHIRURGICO DELLE
CARDIOPATIE CONGENITE
Prof Fabio Miraldi

2. Fetal Echocardiography
 Two forms: basic and extended
 “Four chamber view” the most basic
 Color-flow and spectral Doppler imaging
 M-mode available
 Sensitivity from 60-100%
 Limited by body habitus and fetal age (11
to 14 weeks  transvaginal ultrasound).
16 weeks by abdominal.
 Reported specificity up to 100% in babies
without congenital heart disease

3. IMPROVING
 In 1992, only 8% of infants with congenital
heart disease undergoing cardiac surgery
had a prenatal diagnosis. With fetal echo,
this number rose to 57% in 2002.(Mohan)
 In one study assessing use of early fetal
echo (prior to 14 weeks gestation) in a
high-risk population, sensitivity of 70% and
specificity of 98%
 This resulted in 79% of patients
terminating their pregnancy prior to their
18-20 week follow up.
 Diagnosis of congenital heart disease is
3.5/1000 in II trimester

4. Fetal echocardiography:
advantages
 Counseling
 In some cases, life saving procedures
may be undergone on mother/fetus
before delivery or during pregnancy
 Delivery planning in a special institute
and close to a pediatric cardiac
surgery centre

5. Counseling
 Provide an accurate diagnosis of the
malformation
 Provide a clear and truthful picture of
the prognosis
 Outline management and treatment
options that are available
 Help parents to reach decisions
concerning the form of management
that is best for them

6. Diagnosis and Treatment of Fetal Cardiac Disease
by Mary T. Donofrio, Anita J. Moon-Grady, Lisa K. Hornberger, Joshua A. Copel, Mark
S. Sklansky, Alfred Abuhamad, Bettina F. Cuneo, James C. Huhta, Richard A. Jonas,
Anita Krishnan, Stephanie Lacey, Wesley Lee, Erik C. Michelfelder, Gwen R. Rempel,
Norman H. Silverman, Thomas L. Spray, Janette F. Strasburger, Wayne Tworetzky,
and Jack Rychik
Circulation
Volume 129(21):2183-2242
May 27, 2014

7. Standardized transverse scanning planes for fetal echocardiography include an evaluation of
the 4-chamber view (1), arterial outflow tracts (2, 3), and 3-vessel trachea view (4).
Mary T. Donofrio et al. Circulation. 2014;129:2183-2242

8. Sagittal views of the superior and inferior vena cavae (1), aortic arch (2), and ductal arch (3).
Mary T. Donofrio et al. Circulation. 2014;129:2183-2242

9. Low and high short-axis views of the fetal heart.
Mary T. Donofrio et al. Circulation. 2014;129:2183-2242

11. Transabdominal view of the 4 chambers of the
heart at 13 weeks' gestation.

12. Transvaginal view of atrioventricular
septal defect at 13 weeks' gestation in
a fetus with trisomy 21

13. Transvaginal 3-vessel view of the great arteries at 13 weeks'
gestation. PA, Main pulmonary artery; Ao, aorta; SVC, superior
vena cava; BPA, right branch pulmonary artery.
McAuliffe F.

14. (A) A full examination of the fetal heart may be
obtained by five transverse sections through the
abdomen and chest of the fetus. The first section
shows abdominal situs (B) with the aorta (Ao) to
the left of the spine and the inferior caval vein
(IVC) anterior and to the right. The normal fetal
stomach (St) and heart lie on the left side. The
second section (C) illustrates the four chambers
of the heart with the left atrium (LA) in front of
the spine and the right ventricle (RV) just below
the sternum. The third cut (D) shows the aorta
arising centrally in the heart from the left
ventricle (LV) and the fourth the pulmonary
trunk (PV) arising from the anteriorly placed
right ventricle and crossing to the fetal left over
the ascending aorta (E). The fifth section shows
the anteriorly positioned ductal arch (D) and the
transverse aortic arch (Ao) to be of equal size
traversing back to the fetal spine (F). A normal
variant "three vessel" view is shown with a right
sided aortic arch and persistent left superior
caval vein (LSVC). The trachea (T) can be seen
lying between the aortic (Ao) and ductal (D)
arches (G).
Gardiner.

15. Secondary lesions develop during
following 30-32 gestation weeks
Flow related
theory
Cardiac Embryogenesis
Rational of primary early correction

21. Fetal echocardiography:
advantages
 Counseling
 In some cases, disease modifying or
lifesaving procedures may be
undergone on mother/fetus before
delivery or during pregnancy
 Delivery planning in a special institute
and close to a pediatric cardiac
surgery centre

29. Fetal Aortic Valvuloplasty for Evolving Hypoplastic Left Heart
SyndromeCLINICAL PERSPECTIVE
by Lindsay R. Freud, Doff B. McElhinney, Audrey C. Marshall, Gerald R. Marx, Kevin
G. Friedman, Pedro J. del Nido, Sitaram M. Emani, Terra Lafranchi, Virginia Silva,
Louise E. Wilkins-Haug, Carol B. Benson, James E. Lock, and Wayne Tworetzky
Circulation
Volume 130(8):638-645
August 19, 2014
Copyright © American Heart Association, Inc. All rights reserved.

30. Kaplan–Meier curves depicting intention-to-treat analyses from the date of fetal intervention.
Lindsay R. Freud et al. Circulation. 2014;130:638-645
Copyright © American Heart Association, Inc. All rights reserved.

31. Flow diagram summarizing postnatal management and outcomes for the entire 100-patient
cohort.
Lindsay R. Freud et al. Circulation. 2014;130:638-645
Copyright © American Heart Association, Inc. All rights reserved.

32. Preintervention fetal and neonatal echocardiograms of a patient who underwent technically
unsuccessful fetal aortic valvuloplasty (FAV) and was managed with a univentricular strategy
(A and B), and a patient who underwent technically successful FAV and was managed as
biventricular postnatally (C and D).
Lindsay R. Freud et al. Circulation. 2014;130:638-645
Copyright © American Heart Association, Inc. All rights reserved.

33. Kaplan–Meier curves depicting survival from the time of birth.
Lindsay R. Freud et al. Circulation. 2014;130:638-645
Copyright © American Heart Association, Inc. All rights reserved.

35. Diagnosis and Treatment of
Fetal Cardiac Disease
Circulation
Volume 129(21):2183-2242
May 27, 2014

36. Fetal echocardiography:
advantages
 Counseling
 In some cases, life saving procedures
may be undergone on mother/fetus
before delivery or during pregnancy
 Delivery planning in a special institute
and close to a pediatric cardiac
surgery centre

37. Prostaglandins
The response of the ductus arteriosus
to prostaglandins.
Coceani F, Olley PM.
Can J Physiol Pharmacol. 1973 Mar;51(3): 220-5
Arterial duct

38. Prostaglandins
The response of the ductus arteriosus
to prostaglandins.
Coceani F, Olley PM.
Can J Physiol Pharmacol. 1973 Mar;51(3): 220-5
PGE1
Arterial duct

39. CHD classification
Difetto interatriale
Difetto interventricolare
Dotto arterioso
Canale atrioventricolare
Iperafflusso
polmonare
Coartazione aortica
Stenosi aortica
Stenosi polmonare
Ostruzione degli
efflussi ventricolari
Acianotiche
Tetralogia di Fallot
Atresia tricuspide
Atresia polmonare
Ipoafflusso
polmonare
Trasposizione delle G.A.
Rit. venoso polm. anom. tot.
Truncus arteriosus
Sindrome cuore sx ipoplasico
Mixing
Cianotiche
Cardiopatie congenite

40. Difetto interatriale
Difetto interventricolare
Dotto arterioso
Canale atrioventricolare
Iperafflusso
polmonare
Coartazione aortica
Stenosi aortica
Stenosi polmonare
Ostruzione degli
efflussi ventricolari
Acianotiche
Tetralogia di Fallot
Atresia tricuspide
Atresia polmonare
Ipoafflusso
polmonare
Trasposizione delle G.A.
Rit. venoso polm. anom. tot.
Truncus arteriosus
Sindrome cuore sx ipoplasico
Mixing
Cianotiche
Cardiopatie congenite
Dotto-dipendenze (polmonare o sistemica)
CHD classification
PGE1

41. (
(
(
(
AD
AS
VENTRICOLO AP AO
Parallel circulations: u.h. with single
outlet
PDA
62 1.5
Qp/Qs = 0.5
(
(
(
(
AD
AS
VENTRICOLO AP AO
75 2.0
Qp/Qs = 1.0
PDA
(
(
(
(
AD
AS
VENTRICOLO AP AO
PDA
90 3.5
Qp/Qs = 3.0
Restrictive ASD
neonate
4-5 days
PVRs decrease
PDA closure
SO2 Work load
(X normal)

42. HLHS: rational of perioperative
treatment
NORMALIZE Qp/Qs
Personalized ventilatory treatment
TO INCREASE PVRs:
• decrease FiO2 (21%)
• hypoventilation (PCO2 40 mmHg)
• addition of CO2
• increase insp. Pressures (PEEP)
• avoid anemia (Ht >40%)
• minimal sedation
TO DECREASE PVRs:
• increase FiO2 (100%)
• hyperventilation(PCO2 25-30 mmHg)
• NO
• decrease insp. pressures
• avoid multiple transfusions
• deep sedation

43. Timing chirurgico
Evitare di operare il paziente nel giorno della sua morte!

44. Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of
patients with hypoplastic left heart syndrome with and without prenatal diagnosis
Pediatrics International
Volume 41, Issue 6, pages 728-732, 1 MAR 2002 DOI: 10.1046/j.1442-200x.1999.01154.x

45. Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of
patients with hypoplastic left heart syndrome with and without prenatal diagnosis
Pediatrics International
Volume 41, Issue 6, pages 728-732, 1 MAR 2002 DOI: 10.1046/j.1442-200x.1999.01154.x

46. Has fetal echocardiography improved the prognosis of congenital heart disease? Comparison of
patients with hypoplastic left heart syndrome with and without prenatal diagnosis
Pediatrics International
Volume 41, Issue 6, pages 728-732, 1 MAR 2002 DOI: 10.1046/j.1442-200x.1999.01154.x

47. Has fetal echocardiography improved the prognosis of congenital heart disease?
Comparison of patients with hypoplastic left heart syndrome with and without
prenatal diagnosis

48. Prostaglandin E1 keeps the ductus arteriosus open. If the ductus arteriosus shows
a closing tendency in the hemodynamics of HLHS, the blood flow ejected from the
right ventricle cannot go through the ductus because of ductal stenosis and
systemic circulation cannot be established. Most of the blood flow also goes
through the pulmonary artery instead of the systemic circulation. The patient’s
condition suddenly deteriorates with tachypnea, low cardiac output, peripheral
cyanosis and renal shutdown and blood pressure goes down to shock level. This is
the so-called ‘ductal shock’. Once ductal shock occurs in a patient with HLHS
preoperatively, the patient’s general condition deteriorates and the surgical
outcome may be affected.
In the present study group, there were no patients who had ductal shock in the
prenatally diagnosed group; however, four of 10 patients had ductal shock in the
non-prenatally diagnosed group. Prevention of ductal shock is the most important
issue in preoperative management of patients with HLHS. Thus, we can conclude
that in terms of prevention of ductal shock, prenatal diagnosis of HLHS is quite
beneficial for the patients, although surgical outcomes did not show a significant
difference between the two groups
Ductal shock

52. CHD diagnosis
 Counseling
 Treatment options
-Termination of pregnancy (chromosomal
anomaly, major defects)
-During pregnancy treatment (medical,
interventional)
-Time, type and site of delivery
-Appropriate treatment (which operation)
immediately after birth

53. Diagnosis and Treatment of
Fetal Cardiac Disease
 TGA
 HLHS
 TOF
 EBSTEIN
 Shunt lesions, most
ductal dependent
lesions

55. CONCLUSIONI
 L’ecocardio fetale non ha cambiato
sicuramente l’outcome del
trattamento chirurgico di nessuna
cardiopatia congenita di per sé se non
in quelle trattate con metodica
interventistica in età fetale, ma ha
anticipato/migliorato il trattamento
postnatale influendo quindi sul
successivo decorso

56. Conclusioni
“La selezione naturale
determina la sopravvivenza del più
forte”
(Darwin)
La medicina moderna
deve impegnarsi per
la sopravvivenza e la
qualità di vita del più
debole.