pregnancy in achd

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Massimo Chessa Department of Pediatric Cardiology & Adult with Congenital Heart Disease IRCCS- Policlinico San Donato San Donato Milanese Milano [email protected] PREGNANCY IN ADULT WITH CONGENITAL HEART DISEASE

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Page 1: Pregnancy in ACHD

Massimo Chessa

Department of Pediatric Cardiology

&

Adult with Congenital Heart Disease

IRCCS- Policlinico San Donato

San Donato Milanese – Milano

[email protected]

PREGNANCY

IN ADULT WITH

CONGENITAL HEART DISEASE

Page 2: Pregnancy in ACHD

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

Page 3: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Advances

Diagnosis Therapy

Survival

Page 4: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

The first cause of such

increasing number of patients is

SURGERY

Page 5: Pregnancy in ACHD

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%

Page 6: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Page 7: Pregnancy in ACHD

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.

Page 8: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Page 9: Pregnancy in ACHD

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

Page 10: Pregnancy in ACHD

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

Page 11: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Corrective Surgery…….

Atrial Septal defect

Ventricular Septal Defect

Patent Ductus Arteriosus

…..if treated during

childhood!!!

Page 12: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Residuae Sequelae

Mechanical Electrophysiological

Functional

Page 13: Pregnancy in ACHD

GUCH-PROBLEMS

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

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Page 14: Pregnancy in ACHD

• 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

Page 15: Pregnancy in ACHD

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

Page 16: Pregnancy in ACHD

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

Page 17: Pregnancy in ACHD

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.

Page 19: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Page 20: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Page 21: Pregnancy in ACHD

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

Page 22: Pregnancy in ACHD

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

Page 23: Pregnancy in ACHD

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

Page 24: Pregnancy in ACHD

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

Page 25: Pregnancy in ACHD

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.

Page 26: Pregnancy in ACHD

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.

Page 27: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Pregnancy contraindicated

Page 28: Pregnancy in ACHD

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.

Page 29: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

GENERAL RECOMMENDATIONS

Arrhythmias

Page 30: Pregnancy in ACHD

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

Page 31: Pregnancy in ACHD

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

Page 32: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

GENERAL

RECOMMENDATIONS

Anticoagulation

Page 33: Pregnancy in ACHD

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

Page 34: Pregnancy in ACHD

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

Page 35: Pregnancy in ACHD

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

Page 36: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Page 37: Pregnancy in ACHD

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%

Page 38: Pregnancy in ACHD

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%).

Page 39: Pregnancy in ACHD

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.

Page 40: Pregnancy in ACHD

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.

Page 41: Pregnancy in ACHD

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

Page 42: Pregnancy in ACHD

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?

Page 43: Pregnancy in ACHD

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.

Page 44: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Page 45: Pregnancy in ACHD

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.

Page 46: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

An Italian single centre experience

Page 47: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Page 48: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

An Italian single centre experience

Page 49: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

An Italian single centre experience

Page 50: Pregnancy in ACHD

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

Page 51: Pregnancy in ACHD

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

Page 52: Pregnancy in ACHD

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

Page 53: Pregnancy in ACHD

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

Conclusions

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

Avoid just a single point of view!

Page 54: Pregnancy in ACHD

Patient

Pediatric cardiologist

Gynecologist

Neonatologist

GUCH/ACHD Cardiologist

Department of Pediatric Cardiology & Adult with Congenital Heart Disease

We must act as a team!!

Page 55: Pregnancy in ACHD

Massimo Chessa

Department of Pediatric Cardiology

&

Adult with Congenital Heart Disease

IRCCS- Policlinico San Donato

San Donato Milanese – Milano

[email protected]

Thank you for your attention