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Massimo Chessa
Department of Pediatric Cardiology
&
Adult with Congenital Heart Disease
IRCCS- Policlinico San Donato
San Donato Milanese – Milano
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
Nursing
GUCH Cardiologist
Community Cardiologist
Primary Caregivers
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Advances
Diagnosis Therapy
Survival
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
The first cause of such
increasing number of patients is
SURGERY
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Extraordinary Management Advances Improved
Survival Rate
Year of birth Complex
CHD
Moderate
CHD
Simple
CHD
1940-1959 10% 55% 90%
1960-1979 50% 65% 95%
1980-1989 80% 90% 95%
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Surgery is corrective, if….
…..ventricular function is normal
…..life expetancy is normal!
…..there is no need for tharapeutic
measures during f-up
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Corrective Surgery…….
Atrial Septal defect
Ventricular Septal Defect
Patent Ductus Arteriosus
…..if treated during
childhood!!!
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Residuae Sequelae
Mechanical Electrophysiological
Functional
GUCH-PROBLEMS
Verheugt C L, Heart 2010;96:872-878.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
• Cardiovascular issues: 2-3 times more than general population in subjects over 30 yrs
Hospitalization
Verheugt C L, Heart 2010;96:872-878.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
1966 first successful pregnancy in a pt
With a Starr-Edward mitral valve
……..what about pregnancy
in ACHD more than 40 yrs after ?
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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),
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
From: Abbas Amr E, Lester Steven J, Connolly H: Pregnancy
and the cardiovascular system. International Journal of
Cardiology, 2005; 98: 11
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
HEMODYNAMIC CHANGES
DURING LABOR AND DELIVERY
in blood flow and cardiac output
of blood pressure
of O2 consumption (up to 100%)
UTERINE CONTRACTION
CESAREAN SECTION Hemodynamic changes are influenced by the
type of anaesthesia
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Pregnancy contraindicated
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Infective endocarditis during pregnancy is rare, 0.006%
(1 per 100 000 pregnancies)
GENERAL RECOMMENDATIONS
Infective Endocarditis
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.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
GENERAL RECOMMENDATIONS
Arrhythmias
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
GENERAL RECOMMENDATIONS
Anticoagulation
Hemostatic changes during normal pregnancy
Concentration of coagulation factors
Concentration of fibrogen
Platelet adhesiveness
INCREASED RISK OF THROMBO-EMBOLIC EVENTS
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
GENERAL RECOMMENDATIONS
Anticoagulation
INDICATIONS
Atrial fibrillation
Impaired ventricular function
PAH
Cyanotic Heart Disease
Fontan Circulation
Venous Thrombosis
Pulmonary emboli
Valvular Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
GENERAL
RECOMMENDATIONS
Anticoagulation
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
GENERAL RECOMMENDATIONS
Drugs and fetus
CLASS DRUGS SAFETY
INOTROPIC
AGENTS
Digoxin +
DIURETICS Furosemide +
(not chronic use)
-BLOCKERS Propanolol, atenolol + -
Preterm delivery,
SGA, neonatal
bradycardia and
hypovolemia
CALCIUM
CHANNEL
BLOCKERS
verapamil + -
Uterine blood
flow
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
CLASS DRUGS SAFETY
ANTIARRHTHMIC
AGENTS
Lidocaina,
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
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
GENERAL RECOMMENDATIONS
Delivery
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
One of the most common cardiac complications that has consistently been found
Congestive Heart Failure
with an incidence of 16.7%
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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%).
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Perhaps these risk factors were not previously identified because
of the strong influence of acquired forms of heart disease on
risk factor identification.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
They found an incidence of 9% (7/76 pregnancies) symptomatic RHF.
On univariate analysis, twin pregnancy and BPS were significantly
associated with RHF
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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.
Pathophysiology
RV volume overload? RV compliance? Arrhythmias?
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Based on these findings, the recommendation for prophylactic
pulmonary valve replacement in patients with moderate-to-severe
PR prior to pregnancy should be reconsidered.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Giamberti G, Chessa M, et al. Ann Thorac Surg. 2009
4,9
1,8
9,8
4,3
10,4
22,6
4,2
17,1
3,6
1,2
7,9
11,5
0,0
5,0
10,0
15,0
20,0
25,0
MVR
Resi
dual V
SD
Rees
idua
l ASD
Asc
endig A
o Dila
tatio
n
Pulm
onar
y co
nduit s
teno
sis
PVR
PVS
AVS
/AVR
TVR
Ao
Re.
coar
ctatio
n
Fonta
n
Oth
ers
Reoperations in ACHD are very frequent today
and mostly due to right ventricular outflow
tract lesions.
RVOT problems
38%
Aort a
22%
Sept al def ect
18%
Ot her
22%
RVOT problems
Aort a
Sept al def ect
Ot her
Chessa M, et al. J Cardiovasc Med (Hagerstown). 2006 Sep;7(9):701-5.
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
An Italian single centre experience
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
An Italian single centre experience
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
An Italian single centre experience
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
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
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
Conclusions
Fetal safety or Maternal safety??........
Avoid just a single point of view!
Patient
Pediatric cardiologist
Gynecologist
Neonatologist
GUCH/ACHD Cardiologist
Department of Pediatric Cardiology & Adult with Congenital Heart Disease
We must act as a team!!
Massimo Chessa
Department of Pediatric Cardiology
&
Adult with Congenital Heart Disease
IRCCS- Policlinico San Donato
San Donato Milanese – Milano
Thank you for your attention