Pregnancy in ACHD

Massimo Chessa

Department of Pediatric Cardiology
&
Adult with Congenital Heart Disease

IRCCS- Policlinico San Donato
San Donato Milanese – Milano

massimo.chessa@grupposandonato.it

PREGNANCY
IN ADULT WITH
CONGENITAL HEART DISEASE

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Congenital Heart Disease Is a
continuum
From Fetal Life Until Adulthood

CHILDHOOD TRANSITION ADULTHOOD

0 12 16

Pediatricians Pediatricians GUCH Cardiologist
GUCH Cardiologist Community Cardiologist
Nursing Primary Caregivers


Advances

Diagnosis Therapy

Survival


The first cause of such
increasing number of patients is
SURGERY


Extraordinary Management Advances Improved
Survival Rate

Year of birth Complex Moderate Simple
CHD CHD CHD

1940-1959 10% 55% 90%

1960-1979 50% 65% 95%

1980-1989 80% 90% 95%


More than 75.000 – 100.000 ACHD in Italy
1200-1600 adolescents enter adulthood every year
and require life-long care
More than 85% of infants are expected to reach
adulthood

Chessa M, Cullen S, Deanfield J The care of adult patients with congenital heart
defects: a new challenge. Ital Heart J. 2004 Mar;5(3):178-82.


It is important to outline that
most pts with CHD who have
had their lives transformed by
surgical intervention, had
reparative and not corrective
surgery


Surgery is corrective, if….

…..ventricular function is normal

…..life expetancy is normal!

…..there is no need for tharapeutic
measures during f-up


Corrective Surgery…….

Atrial Septal defect
Ventricular Septal Defect
Patent Ductus Arteriosus

…..if treated during
childhood!!!


Residuae Sequelae

Mechanical
Electrophysiological

Functional


GUCH-PROBLEMS

Verheugt C L, Heart 2010;96:872-878.


Hospitalization
• Cardiovascular issues: 2-3 times more than
general population in subjects over 30 yrs

Verheugt C L, Heart 2010;96:872-878.


1966 first successful pregnancy in a pt
With a Starr-Edward mitral valve

……..what about pregnancy
in ACHD more than 40 yrs after ?


At present, 0.2–4% of all pregnancies in western
industrialized countries are complicated by cardiovascular
diseases.

The spectrum of CVD in pregnancy is changing and
differs between countries.

In western countries maternal heart disease is
now the major cause of maternal death during
pregnancy


This study has shown the possibility that
chronic heart disease among women ospitalised
during pregnancy has increased in severity from
1995–1997 to 2004–2006 in the USA, and also
provided evidence that chronic heart disease in
pregnant women is one of the major
contributors to severe obstetric complications.


J Perinat Med. 2011 May;39(3):251-6

Spectrum of cardiovascular findings during pregnancy and parturition at a
tertiary referral center.
Merz WM, Keyver-Paik MD, Baumgarten G, Lewalter T, Gembruch U.

Cardiac lesions were congenital in 53.1%

Overall cardiac complications: 22

Congestive Heart Failure (n=5),


Additional insult to the
circulatory physiology by
other factors coexisting
together with congenital
heart disease can further
reduce the cardiac reserve in
pregnancy and precipitate
heart failure


From: Abbas Amr E, Lester Steven J, Connolly H: Pregnancy
and the cardiovascular system. International Journal of
Cardiology, 2005; 98: 11


HEMODYNAMIC CHANGES
DURING LABOR AND DELIVERY

in blood flow and cardiac output
of blood pressure UTERINE CONTRACTION

of O2 consumption (up to 100%)

Hemodynamic changes are influenced by the
CESAREAN SECTION
type of anaesthesia


HEMODYNAMIC CHANGES
IN POST-PARTUM

of blood volume
of cardiac output (immediate, secondary to the shift of the
blood from the uterus to the systemic circulation and to the
decreased caval compression with increase of the venous
return, subsequent rapid decrease of cardiac output
in heart rate
of vascular peripheral resistances


Pre-pregnancy counselling : risk estimation

Disease-specific series are usually retrospective and too small to
identify predictors of poor outcome.
Therefore, risk estimation can
be further refined by taking into account
predictors that have been identified in
studies that included larger populations
with various diseases.
Several risk scores have been developed
based on these predictors, of which the
CARPREG risk score is most widely known and
used.


Pre-pregnancy counselling : risk estimation

The Task Force recommends that maternal risk assessment is
carried out according to the modified World Health Organization
(WHO) risk classification.

This risk classification integrates all
known maternal cardiovascular risk factors
including the underlying
heart disease and any other co-morbidity.


Pregnancy contraindicated


GENERAL RECOMMENDATIONS
Infective Endocarditis

Infective endocarditis during pregnancy is rare, 0.006%
(1 per 100 000 pregnancies)

Patients with the highest risk for infective endocarditis are those with a
prosthetic valve or prosthetic material
used for cardiac valve repair, a history of
previous infective endocarditis, and some
special patients with congenital heart
disease.


Arrhythmias


Anticoagulation

Hemostatic changes during normal pregnancy

Concentration of coagulation factors

Concentration of fibrogen

Platelet adhesiveness

INCREASED RISK OF THROMBO-EMBOLIC EVENTS


Anticoagulation

INDICATIONS

Atrial fibrillation
Impaired ventricular function
PAH
Cyanotic Heart Disease
Fontan Circulation
Venous Thrombosis
Pulmonary emboli
Valvular Heart Disease


GENERAL
RECOMMENDATIONS
Anticoagulation


Drugs and fetus

CLASS DRUGS SAFETY
INOTROPIC Digoxin +
AGENTS

DIURETICS Furosemide +
(not chronic use)
-BLOCKERS Propanolol, atenolol +-
Preterm delivery,
SGA, neonatal
bradycardia and
hypovolemia
CALCIUM verapamil +-
CHANNEL Uterine blood
BLOCKERS flow


CLASS DRUGS SAFETY
ANTIARRHTHMIC Lidocaina, +
AGENTS procainamide,
quinidine
Amiodarone -
Neonatal
hypothyroidism
VASODILATATORS Nitroglycerin +-
Volume depletion,
hypotension and fall
in uterine blood
flow
ACE INHIBITORS Captopril, enalapril -
Oligohydramnios,
renal failure, bone
malformation


Delivery

Vaginal delivery is first choice for most patients
- Less blood loss, less infections, lower thromboebolic risk

Indications for caesarian delivery
-Obstetric indications
-Preterm labour in patients on OAC
-Marfan and Aortic dilatation (> 40-45 mm)
-Acute or chronic aortic dissection
-Severe Heart failure
-Severe AS/LVOTO
-Eisenmenger syndrome


One of the most common cardiac complications that has consistently been found

Congestive Heart Failure

with an incidence of 16.7%


The incidence of acute heart failure was the
highest in patients with cardiopathy induced
by hypertensive disorders complicating
pregnancy (80.0%), followed by patients
with PPCM (52.2%), patients with RHD
(27.3%), and patients with CHD (6.9%).


In addition to previously described risk factors
(systemic ventricular EF <40%, baseline NYHA functional class II or cyanosis, left heart obstruction and prior cardiac event
by Siu SC. Circulation. 2001;104:515–521),

the present study found an increased incidence of adverse cardiac events
in pregnant women with depressed subpulmonary ventricular ejection
fraction and/or severe pulmonary regurgitation.


Perhaps these risk factors were not previously identified because

of the strong influence of acquired forms of heart disease on

risk factor identification.


They found an incidence of 9% (7/76 pregnancies) symptomatic RHF.

On univariate analysis, twin pregnancy and BPS were significantly
associated with RHF


Pathophysiology
RV volume overload? RV compliance? Arrhythmias?

Different degrees of chamber enlargement in systemic and
pulmonary circulations have been observed. Whereas subaortic
ventricular size increases by 6%, the right atrium and subpulmonary
ventricle increase by 20%.

Morphological characteristics of an already compromised
subpulmonary ventricle may enhance susceptibility to further
pregnancy-induced volume loading.


Based on these findings, the recommendation for prophylactic
pulmonary valve replacement in patients with moderate-to-severe
PR prior to pregnancy should be reconsidered.


25,0 22,6 Giamberti G, Chessa M, et al. Ann Thorac Surg. 2009
20,0
17,1

15,0
11,5
9,8 10,4
10,0 7,9
4,9 4,3 4,2
5,0 3,6
1,8 1,2
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Reoperations in ACHD are very frequent today
Chessa M, et al. J Cardiovasc Med (Hagerstown). 2006 Sep;7(9):701-5.
and mostly due to right ventricular outflow
tract lesions.
Ot her
22%

RVOT problems
38%
RVOT problems
Aort a
Sept al def ect

Sept al def ect Ot her
18%

Aort a
22%


An Italian single centre experience


Conclusions

 Women with simple CHD or without significant hemodynamic or rythm
impairment may have uncomplicated courses of pregnancy and no impact on the
subsequent cardiac state

 However some lesions, such as ASD II (even when operated), CoA, D/HCMP
and cyanogen defects may present serious problems, due to arrythmias or
systemic or pulmonary hypertension


Conclusions and Advices

• Prepregnancy counselling and evaluation is
mandatory ( physical examination, assesment
of hemodynamic status, functional capacity)

• Stratifications into high, medium and low risk

• Pulmonary hypertension: marked increase of
risk of maternal and fetal mortality


Conclusions and Advices

• Review of medications to avoid drugs that
may be deterious to the fetus

• Anticoagulant drugs for mechanical
prosthetic valves can be associated with
fetal embriopathy

• Small but increased risk of congenital heart
disease in offspring


Conclusions
Fetal safety or Maternal safety??........

Avoid just a single point of view!


We must act as a team!!

Pediatric
cardiologist

Patient

GUCH/ACHD Gynecologist
Cardiologist Neonatologist

Massimo Chessa

Department of Pediatric Cardiology
&
Adult with Congenital Heart Disease

IRCCS- Policlinico San Donato
San Donato Milanese – Milano

massimo.chessa@grupposandonato.it

Thank you for your attention

Pregnancy in ACHD

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