Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge

Avila, Walkiria Samuel; Ribeiro, Veronica Martins; Rossi, Eduardo Giusti; Binotto, Maria Angelica; Bortolotto, Maria Rita; Testa, Carolina; Francisco, Rossana; Hajjar, Ludhmilla Abraão; Miura, Nana

doi:10.5935/abc.20190197

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Original Article • Arq. Bras. Cardiol. 113 (6) • Dec 2019 • https://doi.org/10.5935/abc.20190197 copy

Pregnancy in Women with Complex Congenital Heart Disease. A Constant Challenge

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

Background:

The improvement in surgical techniques has contributed to an increasing number of childbearing women with complex congenital heart disease (CCC). However, adequate counseling about pregnancy in this situation is uncertain, due to a wide variety of residual cardiac lesions.

Objectives:

To evaluate fetal and maternal outcomes in pregnant women with CCC and to analyze the predictive variables of prognosis.

Methods:

During 10 years we followed 435 consecutive pregnancies in patients (pts) with congenital heart disease. Among of them, we selected 42 pregnancies in 40 (mean age of 25.5 ± 4.5 years) pts with CCC, who had been advised against pregnancy. The distribution of underlying cardiac lesions were: D-Transposition of the great arteries, pulmonary atresia, tricuspid atresia, single ventricle, double-outlet ventricle and truncus arteriosus. The surgical procedures performed before gestation were: Fontan, Jatene, Rastelli, Senning, Mustard and other surgical techniques, including Blalock, Taussing, and Glenn. Eight (20,0%) pts did not have previous surgery. Nineteen 19 (47.5%) pts had hypoxemia. The clinical follow-up protocol included oxygen saturation recording, hemoglobin and hematocrit values; medication adjustment to pregnancy, anticoagulation use, when necessary, and hospitalization from 28 weeks, in severe cases. The statistical significance level considered was p < 0.05.

Results:

Only seventeen (40.5%) pregnancies had maternal and fetal uneventful courses. There were 13 (30.9%) maternal complications, two (4.7%) maternal deaths due to hemorrhage pos-partum and severe pre-eclampsia, both of them in women with hypoxemia. There were 7 (16.6%) stillbirths and 17 (40.5%) premature babies. Congenital heart disease was identified in two (4.1%) infants. Maternal and fetal complications were higher (p < 0.05) in women with hypoxemia.

Conclusions:

Pregnancy in women with CCC was associated to high maternal and offspring risks. Hypoxemia was a predictive variable of poor maternal and fetal outcomes. Women with CCC should be advised against pregnancy, even when treated in specialized care centers.

Keywords
Pregnancy; Heart Defects; Congenital/complications Maternal Mortality; Fetal Mortality; Maternal and Fetal Outcomes

Clinical status
Age (years), mean ± dp	16 to 41 (mean 24.5 ± 3.4)
Oxygen saturation (%)	76 - 99 (mean 88.5)
Hemoglobin (mg/dL)	10,5 - 22,0 (mean 14.8)
Hematocrit (%)	32 - 69 (mean 47)
Functional Class (NYHA) (%)	I and II: 35 (79%) pts
	III: 9 (21%) pts
	IV: 0 (0%) pts
Previous surgical repair	34 (77.3%) pts
No previous surgical repair	8 (20%) pts
Hypoxemia (Sat% < 92%)	19 (47.5%) pts

Case	Age (Years)	Specific Cardiac Defects	Surgical repair technique	Sat %	Hb/Ht	Delivery/ Gestational Age/ Newborn Weight/gr
1	18	d-TGA	Senning	99%	13.5/ 39	Vd/ 37/2150
2	20	AP + VSD	Fontan	80%	19.7/ 62	Cs/29/1950
3	21	AP + VSD + APC	no repair	80%	15.0 / 44	Vd/27/1750
4	18	Truncus arteriosus	Rastelli	99%	12.5/38	Cs/40/3360
5	20	AP + VSD + PDA	Rastelli	98%	11.6/ 35	Cs/33/1860
6	19	d-TGA	Jatene	96%	11.2/ 33	Vd/37/2230
7	33	DVSVD + PS	Conduit LV- Ao + RV - PT	98%	12.8/35	Cs/38/2850
8	27	DVEVE + AAVC	no repair	93%	15.9/47	Vd/37/2630
9	18	d-TGA + VSD + PS	Jatene	98%	12.1/ 36	Vd/38/3886
10	32	d-TGA + PS + VSD	BT + Rastelli	98%	10.5/ 32	Vd/38/3210
11	24	DVSVD+dTGA+ PS+VSD	Conduit VE-AO + Rastelli	99%	11.5 / 36	Vd/37/2250
12	19	dTGA+ASD+VSD+ PS	no repair	80%	17.0/54	Miscarriage
13	32	PA	BT + Glenn	76%	17.5/50	Cs/36/1980
14	23	dTGA + VSD	Jatene	87%	12.5/40	Cs/34/2130
15	21	Single Ventricle	Glenn+ Fontan	92%	13.5/42	Cs/31/1400
16	29	DVSVD	BT + Glenn	78%	13.8/49	Cs/29/1250
17	38	Single ventricle	Fontan	82%	16.8/50	Miscarriage
18	27	Single ventricle	Fontan	83%	16.9/50	Cs/28/850g
19	19	TA + VSD + PS	BT + Glenn +Fontan	87%	15.0/47	Vd/37/2030
20	29	PA + VSD	Rastelli + Conduit RV-PT	90%	13.0/42	Cs/37/2350
21	32	AT+ASD+ VSD + PS	Fontan	94%	10.4/32	Cs/35/1800
22	34	Single ventricle	no repair	89%	22.0/69	Abortion
23	29	d-TGA	Senning	93%	11.9/40	Vd/ 39/ 2720
24	30	DVEVE + VSD	Pulmonary truncus banding	89%	17.0/49	Cs/35/1750
25	16	d-TGA	Jatene	96%	12.5/40	Cs/38/3420
26	22	AT	no repair	85%	13.7/40	Cs/32/1150
27	29	d-TGA	Jatene	93%	12.2/39	Cs/38/2460
28	32	DVSVD+d-TGA+PS	Mustard	93%	12.5/45	Cs/36/2240
29	40	d-TGA	Jatene	95%	12.3/42	Cs/37/2570
30	17	DVEV único-E+ ASD+ VSD+PS	no repair	93%	15.9/47	Vd/ 39/ 2720
31	22	DVEV único -E	no repair	94%	12.8/45	Vd/37/2630
32	24	Truncus arteriosus	Conduit valved	93%	12.5/42	Cs/38/3270
33	32	dTGA+ ASD +VSD+ PS	Fontan	91%	13.0/43	Vd/33/1510
34	26	AP + VSD	no repair	87%	21.0/67	Vd/30/1120
35	18	dTGA+ASD+VSD+PS.	Senning	90%	11.8/42	Vd/38/2770
36	19	d-TGA + VSD+ASD+ PS	Conduit LV + Reconstruction atrioventricular	93%	11.8/42	Cs/37/2410
37	20	d-TGA	Jatene	95%	11.9/40	Cs/38/3220
38	41	AP + VSD+PDA	BT + Collateral arterial repair	87%	15.4/45	Cs/28/500
39	31	DVSVD + VSD	Rastelli + Conduit LV-AO	96%	13.4/40	Vd/36/2480
40	1	d-TGA +VSD+PS	Rastelli+ Conduit LV-Ao	90%	11.5/40	Vd/38/3500
41	27	AT + VSD + PS	BT+ Glenn + Fontan	93%	13.8/40	Cs/34/1750
42	24	Truncus arteriosus	Ao + VSD repair + pulmonary graft	94%	11.5/40	Cs/37/2168

Variables No cases	Hypoxemia n = 19	No hipoxemia n = 23	p value	Univentricular n = 16	Bi-ventricular (n = 26)	p value	Operated n = 34	Unoperated n = 8	p value
Unventfull evolution n = 17	3 (15.7%)	14 (60.8%)	< 0.05	4 (25%)	13 (50%)	0.0	14 (41.2%)	3 (37.5%)	1.0
Maternal complications n = 13	9 (47.4%)	4 (17.4%)	< 0.05	7 (43.7%)	6 (23.1%)	0.18	10 (29.4%)	3 (37.5%)	0.68
Maternal death n = 2	2 (10.5%)	0	0.19	0	2 (7.7%)	0.51	1 (2.9%)	1 (12.5%)	0.34
Fetal complications n = 23	18 (94.7%)	5 (21.7%)	< 0.05	12 (75%)	11 (42.3%)	0.06	18 (52.9%)	5 (62.5%)	0.70
Stillbirths n = 7	7 (36.8%)	0	< 0.05	4 (25%)	3 (11.5%)	0.39	3 (8.8%)	4 (50%)	< 0.05
Premature babies n = 17	12 (63.1%)	5 (21.7%)	< 0.0.5	10 (62.5%)	7 (26.9%)	< 0.05	14 (41.2%)	3 (37.5%)	1.0
Mean weight newborns (g)	± 600	± 527	< 0.05	1841 ± 454	2531 ± 496	< 0,05	2246 ± 660	2296 ± 749	0.76

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E-mail: revista@cardiol.br

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[1] Mailing Address: Walkiria Samuel Avila, Intituto de Coração - Cardiopneumologia - Av. Dr. Eneas Carvalho Aguiar, 44. Postal Code 05403-000, São Paulo, SP - Brazil E-mail: walkiria@incor.usp.br

WHO I	Risk no higher of maternal mortality and very low morbidity: uncomplicated, small or mild (pulmonary stenosis; ventricular septal defect; patent ductus); successfully repaired simple lesions (atrial or ventricular septal defect; patent ductus arteriosus; anomalous pulmonary venous drainage).
WHO II	Small increased risk of maternal mortality and morbidity: unoperated atrial or ventricular septal, repaired tetralogy of Fallot, repaired coarctation, atrioventricular septal defect.
WHO III	Significant increased risk of maternal mortality and morbidity: systemic right ventricle (e.g. congenitally corrected transposition, simple transposition after Mustard or Senning repair); Fontan circulation ; cyanotic heart disease; other complex congenital heart disease
WHO IV	Very higher risk of maternal mortality and morbidity or severe morbidity Pulmonary arterial hypertension of any cause; severe systemic ventricular dysfunction; heart failure and LVEF <30%; severe left heart obstruction, severe (re)coarctation; Fontan with any complication.