IMPATTO DELL’ECOCARDIOGRAFIA

FETALE SULL’OUTCOME CHIRURGICO DELLE

CARDIOPATIE CONGENITE

Prof Fabio Miraldi

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

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

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

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

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

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

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

Low and high short-axis views of the fetal heart.

Mary T. Donofrio et al. Circulation. 2014;129:2183-2242

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

Transvaginal view of atrioventricular septal defect at 13 weeks' gestation in

a fetus with trisomy 21

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.

(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.

Secondary lesions develop during following 30-32 gestation weeks

Flow related theory

Cardiac Embryogenesis Rational of primary early correction

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

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.

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.

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.

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.

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.

Diagnosis and Treatment of Fetal Cardiac Disease

Circulation

Volume 129(21):2183-2242

May 27, 2014

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

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

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

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

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

( (

(

(

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)

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

Timing chirurgico

Evitare di operare il paziente nel giorno della sua morte!

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

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

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

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

prenatal diagnosis

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

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

Diagnosis and Treatment of Fetal Cardiac Disease

TGA

HLHS

TOF

EBSTEIN

Shunt lesions, most ductal dependent lesions

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

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.