Integrated pregnancy and perinatal management of complex fetal congenital heart disease: a preliminary retrospective study

Abstract

Background

Congenital heart disease (CHD) is the most prevalent congenital abnormality worldwide and is the primary contributor to neonatal mortality. Obstetricians in China continue to face uncertainties about optimal delivery timing, appropriate delivery mode, perinatal interventions, and postnatal surgical outcomes for fetuses with complex CHD.

Objective

To investigate integrated pregnancy and perinatal management strategies for fetuses diagnosed with complex CHD by prenatal ultrasound in China.

Methods

A retrospective analysis was performed on 123 fetuses with complex CHD diagnosed by prenatal ultrasound at Peking University People’s Hospital between 2016 and 2023.

Results

The mean gestational age at ultrasound diagnosis was 23.7 ± 3.4 weeks. Among the 123 cases, 11 (8.9%) were total anomalous pulmonary venous connection (TAPVC), 14 (11.4%) pulmonary valve abnormalities (PVA), 7 (5.7%) right ventricular double outlet (DORV), 13 (10.6%) aortic arch abnormalities, 69 (56.1%) complete transposition of the great arteries (D-TGA), and 9 (7.3%) other types. Following prenatal counseling, all pregnancies were continued. Among the 72 fetuses undergoing prenatal genetic testing, 9 (9/72) showed chromosomal variants. Fifty-five pregnant women (44.72%) attempted trial of labor, of whom 46 (37.40%) achieved vaginal delivery, including 6 (4.88%) assisted with forceps. Trial of labor failed in 9 cases, requiring cesarean section. In total, 77 pregnancies were delivered by cesarean section, yielding a cesarean rate of 62.6%. The mean gestational age at delivery was 37.9 ± 1.4 weeks, and the mean neonatal birth weight was 3099.0 ± 479.6 g. The mean umbilical arterial blood gas pH was 7.31 ± 0.05. Postnatally, 86 infants (69.9%) received prostaglandin. Average oxygen saturation increased from 72.8 ± 6.0% before alprostadil administration to 80.5 ± 5.0% afterward, a statistically significant improvement (p = 0.000). A total of 114 neonates underwent surgical intervention; 98.2% achieved favorable outcomes, whereas two infants (1.8%) died postoperatively. Approximately 13.0% were anticipated to require secondary surgery.

Conclusion

Pregnancies involving fetuses with complex CHD should avoid preterm birth whenever possible. Complex fetal CHD alone is not an indication for cesarean delivery; the delivery mode should instead be determined by standard obstetric considerations, and individualized management may be adopted according to their opinions and preferences. When both maternal and fetal status remain stable, scheduling delivery at or beyond 39 weeks may be considered. Newborns who present with low postnatal oxygen saturation should receive prostaglandin to keep the ductus arteriosus open, followed by urgent transfer to pediatric cardiac surgery for definitive management. Coordinated, multidisciplinary care supports favorable clinical outcomes.

Supplementary Information

The online version contains supplementary material available at https://doi.org/10.1186/s12884-026-09616-x.

Congenital heart disease (CHD) is the most prevalent congenital abnormality worldwide and remains the primary contributor to neonatal mortality [1]. In China, CHD has been the leading category of birth defects since 2005. Data from the National Maternal and Child Health Surveillance System indicate that, in 2020, CHD occurred at a rate of 17.32 per 1,000 perinatal infants—defined as those from 28 weeks’ gestation through the first 7 days of life. CHD not only accounts for the highest proportion of birth defects in China (26.7%) but also exhibits the most rapid increase in incidence, with the 2011 rate recorded at 3.56 times that in 2000 [2].

CHD encompasses both simple and complex forms, the latter representing approximately 20–30% of affected fetuses [3–6]. Without timely diagnosis and intervention, as many as 69% of newborns with complex CHD die from severe postnatal complications [7]. Advances in prenatal ultrasonography and pediatric cardiac surgery have markedly improved outcomes, particularly for infants diagnosed before birth. Evidence demonstrates that accurate prenatal detection combined with comprehensive perinatal management significantly decreases mortality associated with complex CHD, underscoring the importance of early identification and coordinated care [8].

Despite these advances, obstetricians in China continue to face uncertainties regarding optimal delivery timing, appropriate delivery mode, essential perinatal interventions, and expected postnatal surgical outcomes for fetuses with complex CHD. This study reviews the integrated prenatal and perinatal management of 123 pregnancies complicated by complex CHD at Peking University People’s Hospital, with the aim of informing clinical practice and improving prognostic assessment for affected newborns. And Our study provides data specific to the Chinese healthcare system based on our experience.

Materials and methods

Data source

This study included 123 pregnant women diagnosed with complex fetal CHD by prenatal ultrasound at Peking University People’s Hospital between January 2016 and December 2023. Of these, 112 cases were referrals from hospitals across 24 provinces. The study was approved by the Ethics Committee of Peking University People’s Hospital (Approval No.: 2025PHB407-001), and all participants provided written informed consent.

Methods

Ultrasonography

All 123 fetuses diagnosed with complex CHD underwent detailed ultrasonographic evaluation performed by certified prenatal sonographers at our institution. Examinations were conducted using the Voluson E8 color Doppler system (GE, USA). Gestational age was established based on the last menstrual period; when this information was unavailable, early-pregnancy ultrasound findings were used to correct dating. The standardized prenatal assessment included routine biometric measurements—such as biparietal diameter, head circumference, femur length, and abdominal circumference—as well as Doppler evaluation of umbilical artery hemodynamics. Concurrently, a systematic survey of all fetal organ systems was performed to identify any associated structural anomalies.

Diagnostic criteria for fetal CHD

All cardiac evaluations followed the examination sequence and standard imaging planes outlined in the Chinese Guidelines for Fetal Cardiac Ultrasound Examination, issued by the Ultrasound Physicians Branch of the Chinese Medical Doctor Association. Guided by the segmental diagnostic approach for CHD, sonographers assessed the situs of the visceral atria, atrioventricular alignment, venous connections, and ventriculoarterial relationships in order. Required cardiac views—including the four-chamber view, left and right ventricular outflow tract views, three-vessel–trachea view, short-axis views of the great arteries, and biventricular short-axis view—were systematically obtained [9]. Postnatal echocardiography confirmed the accuracy of prenatal diagnoses in all newborns.

Definition of high-risk complex CHD in the perinatal period

Simple low-risk CHD was defined as an isolated atrial septal defect (ASD), ventricular septal defect (VSD), or patent ductus arteriosus (PDA). High-risk complex CHD included complete transposition of the great arteries (D-TGA), total anomalous pulmonary venous connection (TAPVC), double-outlet right ventricle (DORV), coarctation of the aorta (CoA), interrupted aortic arch (IAA), pulmonary atresia (PA), Ebstein’s anomaly, tricuspid atresia (TA), hypoplastic left heart syndrome (HLHS), hypoplastic right heart syndrome (HRHS), single ventricle (SV), tetralogy of Fallot (TOF), severe pulmonary stenosis (PS, severe PS was diagnosed on echocardiography accompanied by very severe pulmonary stenosis and right ventricular dysfunction), pulmonary valve abnormality (PVA), and related lesions. According to China’s CHD classification system, these correspond to grade III–IV CHD [10].

Prenatal screening and diagnostic workflow

Routine prenatal care included fetal ultrasound and measurement of nuchal translucency (NT) at 11–13 + 6 weeks of gestation, with NT ≥ 0.25 cm considered increased. Before ultrasound detection of CHD, prenatal aneuploidy screening consisted of mid-trimester serological screening (Down syndrome screening) or non-invasive prenatal testing (NIPT). Pregnant women with indications for invasive prenatal diagnosis underwent amniocentesis or umbilical vein puncture after informed consent, followed by G-banded karyotyping, single-nucleotide polymorphism (SNP) microarray, or whole-exome sequencing (WES). Invasive prenatal diagnosis was recommended for all fetuses with complex CHD.

Pregnancies terminated after the diagnosis of complex CHD due to anticipated poor prognosis, financial considerations, or other reasons—following complete counseling and evaluation—were excluded from the statistical analysis.

Pregnancy complications were diagnosed and managed according to relevant national clinical guidelines. Fetal growth restriction (FGR) was defined as estimated fetal weight or abdominal circumference below the 10th percentile for gestational age. Neonates with birth weight below the 10th percentile for gestational age were classified as small for gestational age (SGA) [11].

Coordinated management during pregnancy and the perinatal period

Once complex CHD was identified prenatally, a multidisciplinary team—including specialists from obstetrics, ultrasound, cardiac surgery, neonatology, pediatric cardiac surgery, and the prenatal diagnosis center—provided integrated care throughout pregnancy and around the time of delivery. Severity stratification of the cardiac defect was performed based on imaging characteristics [12]. Expectant parents received comprehensive counseling regarding anticipated perinatal risks, available interventions, and postnatal treatment pathways, together with individualized prognostic interpretation.

For individuals without signs of impending preterm birth, cervical length surveillance was conducted every 2–4 weeks, and pregnancy complications were managed proactively. Fetal growth was closely monitored to detect FGR. Care plans were customized according to each patient’s clinical profile. At delivery, neonatologists were present to provide immediate support, and coordinated obstetric–pediatric management ensured effective stabilization of the newborn. Infants requiring surgical intervention were transferred promptly to pediatric cardiac surgery for definitive treatment. All surgeries were performed at the same center and by the same surgical teams in Fuwai Hospital. All newborns underwent ultrasound examinations after birth. For complex fetal CHD, the consistency rate between prenatal and postnatal ultrasound diagnoses has remained 100% for our study.

Postnatal follow-up was performed via telephone and outpatient visits. In collaboration with pediatric cardiac surgeons from Fuwai Hospital, Chinese Academy of Medical Sciences, neonatal prognosis was monitored for 3–72 months. A favorable neonatal prognosis was defined as age-appropriate growth and neurocognitive development, absence of cardiac dysfunction, neurological sequelae, and other severe comorbidities. Poor prognosis was defined as the occurrence of any of the following outcomes: neonatal death, refusal of surgical intervention due to prematurity or other reasons, inability to undergo surgery secondary to medical complications, and loss to follow-up.

Statistical methods

Statistical analyses were conducted using SPSS 22.0 software (IBM, Armonk, USA). All continuous variables met normality assumptions and are presented as mean±standard deviation. Group comparisons were performed using independent-samples t-tests. Categorical variables are presented as counts and percentages, with group comparisons conducted using χ² tests. All tests were two-tailed, with P < 0.05 considered statistically significant. The normality test was performed on the data, and non-parametric tests were used for non-normally distributed data when necessary.

Results

General conditions of the study population

Between 2016 and 2023, 123 neonates with complex CHD were delivered at our hospital. During this period, a total of 22,423 deliveries occurred, corresponding to a complex CHD live-birth rate of 0.55% (123/22,423). The mean maternal age was 33.0 ± 4.1 years, with 43 women (35.0%) aged > 35 years and 8 (6.5%) aged > 40 years. The mean gravidity was 2.3 ± 1.3 (including the index pregnancy), and the mean parity was 1.4 ± 0.6 (including the index delivery). None of the women had a previous pregnancy affected by fetal CHD.

The most frequent pregnancy complications or comorbidities were gestational diabetes mellitus (34.1%, 42/123), hypertensive disorders of pregnancy (14.6%, 18/123), twin pregnancy (12.2%, 15/123), thyroid disease (7.3%, 9/123), and FGR (4.9%, 6/123). Detailed data are presented in Table 1.

Table 1.

Pregnancy complications of pregnant women with fetal CHD

CHD Type	Number	IVF-ET	Twin pregnancy			GDM	HBP	Thyroid Disease	FGR	Others
			DCDA	MCDA	MCMA
TAPVC	11 (8.9%)	1 (3.4%)	0	0	0	3 (7.1%)	1 (5.6%)	0	0	0
PVA	14 (11.4%)	1 (3.4%)	1 (7.7%)	1	0	3 (7.1%)	3 (16.7%)	0	0	1 (12.5%)A
DORV	7 (5.7%)	3 (10.3%)	0	0	0	1 (2.4%)	0	1 (11.1%)	0	0
COA	13 (10.6%)	2 (6.9%)	2 (15.4%)	0	0	3 (7.1%)	1 (5.6%)	1 (11.1%)	3 (50.0%)	0
D-TGA	69 (56.1%)	20 (69.0%)	10 (76.9%)	0	1	28 (66.7%)	8 (44.4%)	5 (55.6%)	3 (50.0%)	3 (37.5%)B
Other types	9 (7.3%)	2 (6.9%)	0	0	0	4 (9.5%)	5 (27.8%)	2 (22.2%)	0	4 (50.0%)C
Total	123	29 (23.6%)	13 (10.6%)	1(0.8%)	1(0.8%)	42 (34.1%)	18 (14.6%)	9 (7.3%)	6 (4.9%)	8 (6.5%)

CHD Congenital heart disease, IVF-ET in vitro fertilization–embryo transfer, GDM gestational diabetes mellitus, HBP hypertensive disorders of pregnancy, FGR fetal growth restriction, DCD Adichorionic diamniotic, MCDA monochorionic diamniotic, MCMA monochorionic monoamniotic, TAPVC total anomalous pulmonary venous connection, PVA pulmonary valve abnormality, DORV double outlet of the right ventricle, CoA coarctation of the aorta, D-TGA complete transposition of the great arteries

A: One woman had a history of pulmonary valve stenosis surgery. B: One woman had antiphospholipid syndrome (APS), and two had immune thrombocytopenia (ITP). C: One woman had APS, one had ITP, and two had kidney disease

Ultrasonic findings

The mean gestational age at prenatal ultrasound diagnosis was 23.7 ± 3.4 weeks. Detailed sonographic diagnoses are summarized in Table 2.

Table 2.

Prenatal ultrasonic diagnosis of pregnant women with fetal CHD

Category	Number (%)	Sub-types	Number (%)	Number of Combined Intracardiac Malformations (%)	Number of Combined Extracardiac Malformations (%)
TAPVC	11 (8.9%)	Supracardiac	4 (36.4%)		1 pleural effusion (25.0%)
		Infracardiac	5 (45.5%)	1 VSD(20.0%)
		Intracardiac	2 (18.2%)
PVA	14 (11.4%)	PA/IVS	6 (42.9%)
		PA/VSD	1 (7.1%)
		Severe PS	7 (50.0%)
DORV	7 (5.7%)				1 case of left hand polydactyly and 1 case of right hand syndactyly (28.6%)
COA	13 (10.6%)	COA	10(76.9)	6VSD, 1ASD, total 7 cases(70.0%)
		IAA	3 (23.1%)	2VSD(66.7%)
D-TGA	69 (56.1%)	D-TGA	69 (100%)	14 VSD, 2 restricted foramen ovale (23.2%)	2 cases of hypospadias, 1 case of accessory ear, 1 case of choroid plexus cyst (5.8%)
Other types	9 (7.3%)	TOF	5 (55.6%)
		Cor triatriatum	1 (11.1%)		1 case of small gallbladder (100.0%)
		Anomalous pulmonary venous drainage	2 (22.2%)
		Ventricular aneurysm	1 (11.1%)
Total	123 (100%)			26 (21.1%)	8 (6.5%)

TAPVC Total anomalous pulmonary venous connection, PVA pulmonary valve abnormality, PA pulmonary atresia, PA/IVS pulmonary atresia with intact ventricular septum, PS pulmonary stenosis, DORV double outlet of the right ventricle, CoA coarctation of the aorta, IAA interrupted aortic arch, TGA transposition of the great arteries, D-TGA complete transposition of the great arteries, VSD ventricular septal defect, ASD atrial septal defect, TOF tetralogy of Fallot

Prenatal screening and diagnosis of fetuses with complex CHD

Comprehensive prenatal screening and diagnostic data for pregnant women carrying fetuses with complex CHD are summarized in Table 3. The mean gestational age at invasive diagnostic procedures was 23.7 ± 3.1 weeks. Among the 72 fetuses that underwent invasive prenatal testing, 9 (9/72) exhibited chromosomal variants. Of these, 3 cases (3/9) had abnormal karyotypes and 6 (6/9) showed SNP microarray abnormalities; all identified variants were non-pathogenic. All patients with identified genetic variants received professional genetic counseling, and the potential long-term outcomes for the affected children were fully discussed with the families. Detailed findings are presented in Table 3.

Table 3.

Prenatal screening and diagnosis of pregnant women with fetal CHD

CHD Type	Number(%)	AbnormalNT	Mid-trimester Serological Screening	NIPT	Puncture	Karyotype	SNP	WES	Number of Abnormal Cases
TAPVC	11 (8.9%)	0	7 (17.5%) B	3 (4.2%)	7 (9.7%)	2 (3.3%)	7 (10.8%)	0	1 (1.4%)D
PVA	14 (11.4%)	0	2 (5.0%)	10 (13.9%)	8 (11.1%)	8 (13.1%)	6 (9.2%)	2 (28.6%)	0
DORV	7 (5.7%)	0	2 (5.0%)	4 (5.6%)	4 (5.6%)	3 (4.9%)	4 (6.2%)	0	2 (2.8%)E
COA	13 (10.6%)	0	3 (7.5%)	9 (12.5%)	7 (9.7%)	7 (11.5%)	7 (10.8%)	0	0
D-TGA	69 (56.1%)	3(4.3%)A	23 (57.5%) C	43 (59.7%)	40 (55.6%)	35 (57.4%)	38 (58.5%)	5 (71.4%)	5 (6.9%)F
Other Types	9 (7.3%)	0	3 (7.5%)	3 (4.2%)	6 (8.3%)	6 (9.8%)	3 (4.6%)	0	1 (1.4%)G
Total	123 (100%)	3 (2.4%)	40 (32.5%) 36 cases with low risk	72 (58.5%) All with low risk	72 (58.5%)	61 (49.6%)	65 (52.8%)	7 (5.7%)	9 (12.5%)

CHD Congenital heart disease, TAPVC total anomalous pulmonary venous connection, PVA pulmonary valve abnormality, DORV double outlet of the right ventricle, CoA coarctation of the aorta, D-TGA complete transposition of the great arteries, NT nuchal translucency, NIPT non-invasive prenatal testing, SNP single nucleotide polymorphism microarray, WES whole exome sequencing

A: Three cases of increased NT were 2.8 mm, 3.1 mm, and 3.2 mm. B: One case with borderline risk on mid-trimester serological screening. C: Three cases (4.3%) with abnormal mid-trimester serological screening; two borderline-risk cases proceeded to NIPT, and one case was high-risk for trisomy 21. D: One case with karyotype 46, XN, add (15) p12. E: One case with karyotype 46, XN, inv(1)(p12q12),2p24.3; one case with SNP-confirmed uniparental disomy of chromosome 2. F: Five cases with SNP abnormalities. G: One failed karyotype culture, and one case with 45, XN, rob(14;22)(q10;q10)

Perinatal conditions, surgical outcomes, and prognosis

Among the 123 pregnant women carrying fetuses with complex CHD, 55 (44.72%) underwent a trial of labor, resulting in 46 successful vaginal deliveries (37.40%), including 6 (4.88%) assisted with forceps. Nine women required intrapartum conversion to cesarean section due to suspected fetal distress (4/9), labor arrest (2/9), intrapartum fever (2/9), or failed induction (1/9).

Overall, 77 deliveries occurred by cesarean section. Beyond the intrapartum indications noted above, primary indications included triple nuchal cord loops (1/68, 1.47%), contracted pelvis (2/68, 2.94%), malpresentation (4/68, 5.88%), severe maternal comorbidities (7/68, 10.29%), twin pregnancy (15/68, 22.06%), prior uterine scar (18/68, 26.47%), and maternal request due to fetal CHD (21/68, 30.88%). The total cesarean rate was 62.6%. During the same period, the overall institutional cesarean rate was 40.19%. Excluding maternal-request cesareans for fetal CHD, the adjusted rate for this cohort was 45.1%, not significantly different from the institutional average (P = 0.270).

Fourteen cases (14/123, 11.38%) exhibited intrapartum fetal heart rate abnormalities, including moderate–severe variable decelerations (12/14), tachycardia with absent variability (1/14), and prolonged deceleration (1/14). Twelve infants (12/123, 9.76%) were delivered in grade 3 meconium-stained amniotic fluid. The mean umbilical arterial pH at birth was 7.31 ± 0.05. The neonatal asphyxia rate for vaginal delivery hospital-wide during the same period was 2.40%, compared with 2.44% (3 cases) among vaginal deliveries in the CHD cohort, with no significant difference (P = 0.974).

A neonatologist attended all deliveries. Following initial stabilization, ambient-air oxygen or positive-pressure oxygen was provided as clinically indicated. Alprostadil infusion (6.00–13.00 ng/min/kg) was initiated when ductal patency support was required. One preterm infant (31 weeks) additionally received pulmonary surfactant. A total of 86 neonates received prostaglandin therapy. Mean oxygen saturation increased significantly from 72.8 ± 6.0% before alprostadil to 80.5 ± 5.0% after treatment (P = 0.000). Detailed perinatal, postoperative, and follow-up outcomes are summarized in Table 4.

Table 4.

Perinatal characteristics, neonatal surgical management, and follow-up outcomes in complex fetal CHD

CHD Type	N	Gestational Age at Delivery (Weeks)	Mode of Delivery (N)	Mode of Delivery	Gender (Male/Female)	Birth Weight (g)	SGA (N)	Alprostadil Infusion (N)	Birth Oxygen Saturation(%)	Oxygen Saturation at Transfer (%)	Number of Surgeries (N)	Postnatal Surgery Time (Days)	Possible Need for Reoperation (N)	Number of Adverse Outcomes (%)	Follow-up Time (Months)
TAPVC	11	38.5 ± 1.1	7CS 4VD	63.6%	8:3	3322.7 ± 353.2	0	6(54.5%)	69.9 ± 9.6	82.0 ± 3.4	11(100.0%)A	12.3 ± 10.2 (1–27)	0	1(9.1%) G	15.8 ± 13.2 (5–48)
PVA	14	37.7 ± 1.0	9CS 5VD	64.3%	8:6	3178.6 ± 520.1	1 (7.1%)	10(71.4%)	75.6 ± 4.6	84.3 ± 3.4	12(85.7%)B	15.3 ± 7.5 (2–49)	5 (35.7%)	1 (7.1%) H	11.5 ± 4.3 (6–18)
DORV	7	38.4 ± 1.5	3CS 1FD 3VD	42.9%	4:3	3315.7 ± 540.2	0	6(85.7%)	76.1 ± 2.8	84.0 ± 3.3	7(100.0%)C	30.9 ± 30.4 (9–87)	0	0	10.1 ± 2.9 (6–12)
COA	13	37.4 ± 2.4	11CS 2VD	84.6%	6:7	2630.0 ± 489.6	0	6(46.2%)	75.4 ± 2.8	82.7 ± 3.1	11(84.6%)D	13.7 ± 12.2 (7–47)	4 (30.8%)	2(15.4%) I	12.8 ± 7.4 (3–30)
D-TGA	69	38.1 ± 1.4	41CS 24VD 4FD	59.4%	52:17	3216.1 ± 537.8	3 (4.3%)	55(79.7%)	72.4 ± 6.4	79.1 ± 6.4	66(95.7%)E	10.8 ± 18.3 (1-120)	5(7.2%)	3 (4.3%) J	18.8 ± 17.3 (4–72)
Other types	9	37.2 ± 1.2	6CS 2VD 1FD	66.7%	1:8	2931.1 ± 436.4	0	3(33.3%)	77.7 ± 4.2	85.0 ± 3.2	7(77.8%)F	79.2 ± 88.3 (4-180)	2(22.2%)	1(11.1%) K	13.8 ± 5.9 (7–24)
Total/Average	123	37.9 ± 1.4	40VD 6FD 77CS	62.6%	79:44	3099.0 ± 479.6	4 (3.3%)	86 (69.9%)	72.8 ± 6.0	80.5 ± 5.0	114(92.7%)	27.0 ± 26.6 (1-180)	16(13.0%)	8(6.5%)	13.8 ± 8.5 (3–72)

CHD Congenital Heart Disease, TAPVC Total Anomalous Pulmonary Venous Connection, PVA Pulmonary Valve Abnormality, DORV Double Outlet Right Ventricle, CoA Coarctation of the Aorta, D-TGA complete transposition of the great arteries, CS Cesarean Section , VD Vaginal Delivery, FD Forceps Delivery, SGA Small for Gestational Age

A: All infants underwent definitive TAPVC repair. B: Seven infants received transthoracic pulmonary valve balloon dilation combined with a Blalock–Taussig (BT) shunt; one underwent a modified BT shunt alone; and four underwent isolated transthoracic pulmonary valve balloon dilation. C: Three infants underwent an arterial switch operation (ASO); two underwent ASO combined with an intra-tunnel procedure; one underwent ASO with arch plasty; and one received isolated intra-tunnel repair. D: Surgical procedures included arch plasty (2 cases), arch plasty with VSD repair (1 case), arch coarctation correction (1 case), CoA resection with end-to-end anastomosis (4 cases), interrupted aortic arch repair (1 case), mid-arch interruption repair with VSD closure (1 case), and mid-arch interruption repair with pulmonary artery banding (1 case). E: Fifty-seven infants underwent ASO; seven underwent ASO with VSD repair; one received an intra-tunnel repair; and one received a BT shunt. F: Two infants underwent complete TOF repair; one underwent BT shunt plus PDA ligation plus cor triatriatum repair; one received ASO; and one underwent CoA repair with arch plasty and correction of anomalous pulmonary artery origin. G: One case resulted in postoperative mortality. H: One case was lost to follow-up. I: One preterm infant whose family declined surgery was lost to follow-up, and one additional case was lost to follow-up. J: Two infants developed NEC, and one preterm infant was lost to follow-up. K: One infant required re-operation and subsequently died after the family discontinued treatment

There were 11 cases of TAPVC, the individual Apgar score information was provided in the supplementary materials. One newborn, delivered by cesarean section at 36 + 3 weeks due to pleural effusion, had an Apgar score of 3 at 1 min. After resuscitation, ambient-air oxygen, and thoracentesis with drainage of 200 mL of pleural fluid, Apgar scores improved sequentially to 5 at 5 min, 7 at 10 min. Eight cases were delivered between 39 + 0 and 40 + 0 weeks, all with favorable prognoses. One infant delivered at 37 weeks died postoperatively.

There were 14 cases of PVA, with detailed intrapartum characteristics summarized in Table 4. One newborn with PA/VSD, a twin delivered by cesarean section at 37 + 0 weeks with a birth weight of 2140 g, had followed initial resuscitation and ambient-air oxygen. The family declined further treatment, and the infant was taken home and subsequently lost to follow-up. Six cases were delivered between 39 + 0 and 39 + 4 weeks, and one preterm infant delivered at 36 + 5 weeks due to twin preterm labor also had a good prognosis.

There were seven newborns with DORV. Four infants were delivered between 39 + 2 and 40 + 3 weeks, and one preterm infant born at 36 + 5 weeks due to premature rupture of membranes (PROM) also had a favorable prognosis.

Among the 13 newborns with CoA. Four infants were delivered after 39 weeks (39 + 1–40 + 0 weeks), all with good prognoses. One patient delivered at 38 + 3 weeks of gestation. Postnatal evaluation of the newborn indicated the need for further surgical intervention, but the family declined surgery. Two infants were preterm: one born at 31 + 4 weeks in a twin pregnancy who had a favorable outcome, and another born at 34 + 2 weeks who was lost to follow-up after the family declined surgery.

There were 69 cases of D-TGA, the individual Apgar score information was provided in the supplementary materials. Thirty infants were delivered between 39 + 0 and 41 + 0 weeks, all with good prognoses. Four preterm infants (34 + 3–36 + 6 weeks) were delivered due to twin pregnancy or PROM; one infant delivered at 34 + 3 weeks was lost to follow-up, while the others had good outcomes. Two infants developed necrotizing enterocolitis (NEC)(delivered at 37 + 1 and 38 + 4 weeks) and were lost to follow-up after being transferred for further treatment.

There were nine newborns with other forms of complex CHD. One preterm infant was delivered by cesarean section at 35 + 6 weeks due to breech presentation, severe preeclampsia, and PROM. Despite initial surgery, the family discontinued treatment, and the infant was later found deceased on follow-up. Another preterm infant delivered at 36 + 3 weeks had a good prognosis. One full-term infant delivered at 39 + 2 weeks also had a favorable outcome.

There was a significant difference in the rate of poor prognosis among different delivery gestational age groups (χ² = 13.31 df = 2, P = 0.0013). Specifically, the rate of poor prognosis in the ≥ 39 weeks’ group was zero (Table 5). Due to the small sample size and the heterogeneity of CHD in each group, no intergroup comparisons were performed.

Table 5.

Relationship between delivery gestational age and poor prognosis

CHD Type	N	Gestational Age at Delivery						P
		< 37 weeks		37–38 + 6 weeks		≥ 39 weeks
		poor prognosis(N)	Total(N)	poor prognosis(N)	Total(N)	poor prognosis(N)	Total(N)
TAPVC	11	0	1	1	2	0	8
PVA	14	0	1	1	7	0	6
DORV	7	0	1	0	2	0	4
COA	13	1	2	1	7	0	4
D-TGA	69	1	4	2	35	0	30
Other types	9	1	2	0	6	0	1
Total	123	3(27.3%)	11(8.9%)	5(8.5%)	59(48.0%)	0(0%)	53(43.1%)	0.0013

CHD Congenital Heart Disease, TAPVC Total Anomalous Pulmonary Venous Connection, PVA Pulmonary Valve Abnormality, DORV Double Outlet Right Ventricle, CoA Coarctation of the Aorta, D-TGA complete transposition of the great arteries

Discussion

Complex CHD remains a major threat to child health in China. Variability in diagnostic capabilities, uneven development of multidisciplinary care, and limited public awareness contribute to gaps in prenatal CHD screening in certain regions, resulting in missed opportunities for timely postnatal intervention. Although both detection rates and diagnostic precision for prenatal CHD have improved substantially in recent years, the pathways for counseling, perinatal decision-making, and postnatal management following prenatal CHD detection are still not well defined. More than half of fetuses diagnosed with CHD undergo pregnancy termination, highlighting a significant disconnect between secondary and tertiary prevention efforts. Consequently, fetuses diagnosed with complex CHD require precise prenatal characterization, coordinated perinatal care, and an efficient postnatal referral system to optimize outcomes.

Early, targeted intervention has been consistently shown to enhance survival in children with CHD. Infants diagnosed only after birth frequently arrive in critical condition—often with profound hypoxemia, acidosis, or heart failure—which significantly increases perioperative risk. In contrast, integrated care models that bridge prenatal diagnosis with planned postnatal treatment provide valuable lead time for clinical preparation, enabling selection of optimal surgical strategies, reducing perioperative mortality, and improving overall prognosis [13, 14]. Despite these advances, uncertainty persists among some obstetric providers regarding best practices for managing pregnancies complicated by complex CHD. The present study aims to inform clinical practice and improve prognostic evaluation for affected newborns. Furthermore, we intend to provide practical experience and further clarify the optimal gestational age and mode of delivery for such cases within the context of the Chinese healthcare system.

Ultrasonic diagnosis of fetal complex CHD

Despite substantial advances in prenatal imaging, the ultrasonic diagnosis of complex CHD remains challenging. A meta-analysis by van Velzen et al. reported a prenatal detection rate of 45.1% (95% CI: 33.5%–57.0%) for CHD [15]. The incidence of complex CHD in our hospital was 0.55%, a figure likely influenced by its role as a regional referral center, which receives a high volume of pregnancies complicated by suspected cardiac anomalies. In this study, postnatal echocardiographic reassessment demonstrated high concordance with prenatal ultrasound findings, attributable to our institution’s routine use of combined-section sonographic techniques. These results underscore the reliability, clinical value, and necessity of prenatal ultrasonography for diagnosing complex CHD.

Selection of gestational age for delivery of fetal complex CHD

The Expert Consensus on Perinatal Diagnosis and Clinical Management of Cardiac Birth Defects in China recommends delivery between 38 and 39 weeks for grade IV fetal complex CHD [16]. However, other domestic literature offers no unified standard for the optimal delivery timing in grade III–IV cases. In practice, some clinicians may opt for early delivery, often due to risk aversion or limited experience, even when maternal and fetal conditions are stable. Such premature delivery can inadvertently increase the complexity of subsequent surgical management.

The Obstetrics and Gynecology Branch of the Chinese Medical Association advises that elective cesarean sections be scheduled after 39 weeks [17]. Since implementing our integrated fetal CHD management program in 2016, we have prioritized prolonging gestation whenever safely feasible. Delivery decisions have been based strictly on obstetric indications, allowing most pregnancies to continue beyond 39 weeks. This approach supports improved neonatal pulmonary maturity and facilitates safer, more effective cardiac surgery. In our cohort, two preterm infants demonstrated poor condition after birth, and their families declined further intervention; additional cases delivered between 37 + 0 and 38 + 6 weeks developed NEC, requiring stabilization or surgical treatment prior to cardiac repair.

Routine assessment of cervical length during pregnancy is therefore essential to reducing preterm birth risk. For patients carrying fetuses with complex CHD, planned admission after 39 weeks—based on cervical readiness—can increase the likelihood of successful induction, while minimizing the challenges associated with emergency perinatal management. This strategy reduces supports timely progression to cardiac surgery. Equally important is advanced coordination with referral centers to ensure immediate postnatal transfer to institutions equipped for pediatric cardiac surgery. Our center’s experience offers a practical template for hospitals without in-house pediatric cardiac services, enabling wider adoption of an organized referral system.

Selection of delivery mode for fetal complex CHD

Cesarean section rates in China remain high overall, averaging 41.1% [18], with even higher rates observed among pregnancies complicated by fetal complex CHD. Maternal anxiety, concerns regarding neonatal outcomes, and limited clinician experience at some centers often lead to unnecessary cesarean delivery out of fear that CHD may compromise intrapartum tolerance. However, because the fetal foramen ovale and ductus arteriosus remain patent in utero, pulmonary circulation is minimal, and systemic oxygenation relies primarily on placental gas exchange. Consequently, fetuses with D-TGA, DORV, PVA, CoA, or TAPVC generally maintain circulatory physiology comparable to that of healthy fetuses, with similar tolerance to uterine contractions and no inherently increased risk of intrapartum distress.

Our findings indicate that most fetuses with complex CHD can safely undergo vaginal or forceps-assisted delivery, with no significant differences in cesarean section rates or neonatal asphyxia rates compared with the general obstetric population. Although a small number of vaginally delivered newborns exhibited fetal heart rate deceleration or mild asphyxia, these conditions improved rapidly following standard neonatal resuscitation and did not adversely affect subsequent surgical management or prognosis. Cesarean delivery should therefore be reserved for standard obstetric indications or clear evidence of intrauterine compromise, not for CHD alone. For pregnant women with significant anxiety, individualized management may be adopted according to their opinions and preferences.

For pregnancies with stable maternal and fetal status, vaginal delivery may better facilitate neonatal respiratory adaptation, support early establishment of the gut microbiome, while also reducing unnecessary cesarean sections. Increasing awareness among both patients and obstetric providers regarding the relative safety and advantages of vaginal birth in appropriate cases is essential for optimizing delivery planning and improving overall perinatal care for complex CHD.

Integrated perinatal management for fetuses with complex CHD

Published evidence indicates that when complex CHD is identified prenatally, coordinated evaluation by ultrasound specialists, obstetricians, and pediatric cardiac surgeons is essential for determining the appropriate Level of Care (LOC), with management strategies tailored accordingly [19]. In this cohort, all patients received multidisciplinary consultations throughout pregnancy. Serial assessment of cervical length, continuous fetal monitoring, and vigilant screening for preterm labor and FGR were implemented to minimize preventable risks and optimize neonatal outcomes.

Perinatal plans were individualized, with a strong emphasis on scheduling delivery during daytime hours on weekdays to ensure adequate staffing and streamlined referral pathways. To prevent unanticipated labor, cervical examinations were recommended after 38–39 weeks. When cervical ripeness was confirmed, induction was initiated after 39 weeks; if unfavorable, induction proceeded at 40 weeks. Before delivery, neonatologists were notified, transport teams were placed on standby, and transfer arrangements were secured. Immediately following birth, infants were moved to a cardiac-capable neonatal unit for coordinated management by obstetrics, neonatology, and pediatric cardiac surgery teams.

After delivery, infants underwent clinical evaluation and continuous oxygen saturation monitoring. All newborns with complex CHD received pulse oximetry at birth. Medications such as alprostadil, infusion supplies, and neonatal transport equipment were prepared in advance, and beds were reserved at referral centers. For neonates exhibiting hypoxemia, prostaglandin therapy was initiated to maintain ductal patency and stabilize them for subsequent surgical intervention [18]. In this study, 69.9% of affected infants required alprostadil, and oxygen saturation significantly improved after administration (p = 0.000). These findings reinforce that maintaining ductal patency in hypoxemic newborns with complex CHD is critical for achieving adequate oxygenation and enabling safe transfer and operative management. Due to the limited sample size, only a simple comparison was performed. multivariable analysis will be more conducive to the validity of need for alprostadil in future studies with a larger sample size.

In contrast, primary healthcare institutions often lack the capability for precise prenatal ultrasound diagnosis and on-site pediatric cardiac surgical resources. Accordingly, timely referral to a tertiary hospital is recommended for integrated perinatal management once fetal CHD is suspected or confirmed. With the advancement of digital healthcare, online outpatient consultation has become increasingly accessible. We have therefore established a streamlined referral pathway for all pregnant individuals with a prenatal diagnosis of fetal CHD, enabling convenient specialist consultation and standardized referral management for patients transferred from primary medical facilities.

Prognosis of newborns with complex CHD

Previous studies report high mortality among infants with complex CHD, with an overall survival rate near 80%, underscoring the need for rapid referral to specialized pediatric cardiac centers [8]. Among the 123 newborns in this study, all received the indicated postnatal surgical or interventional treatments. Postoperative follow-up revealed favorable outcomes in 98.2% of cases, with only two deaths—one resulting from the family’s decision to discontinue care. Approximately 13.0% of infants were expected to require additional operations. These findings highlight that, in the absence of other serious complications, surgical management offers an excellent prognosis for most infants with complex CHD.

For families who declined surgery, financial strain, time commitments, and insufficient understanding of the long-term care process may have influenced decision-making. Thus, prenatal counseling must be individualized and comprehensive, ensuring that families receive clear explanations of both the challenges and the substantial potential benefits of treatment, enabling them to choose the option most supportive of maternal and neonatal health.

Limitations

This study is a single-center retrospective design and includes a relatively small cohort of fetuses with complex CHD, which may limit the generalizability of the findings. Also we excluded cases with termination of pregnancy after prenatal diagnosis of CHD, which may lead to selection bias. So our study is primarily descriptive and exploratory. In addition, neonatal follow-up remains confined to the short- to medium-term, preventing a comprehensive evaluation of long-term outcomes. And owing to variations in the timing of delivery across pregnant women, the duration of follow-up varied substantially, and long-term neurological follow-up data for the children remain unavailable. For the small sample size, further statistical analysis is not powered for comparing outcomes by CHD subtype, surgical timing, or risk category. The cohort remains highly heterogeneous, and our study is hardly powered for lesion-specific comparisons. And we acknowledge that our conclusion was supported by observational data rather than definitive evidence. Therefore, multicenter, large-scale prospective studies with extended follow-up are required to more accurately assess long-term prognosis in affected newborns.

In summary, for fetuses diagnosed with complex CHD through prenatal ultrasound, coordinated perinatal management significantly enhances maternal and neonatal outcomes. Delivery mode should better be based on obstetric indications, as complex CHD alone does not justify cesarean delivery, individualized management may be adopted according to their opinions and preferences. When maternal and fetal conditions are stable, planned delivery at ≥ 39 weeks may be considered. Postnatally, infants with reduced oxygen saturation benefit from prostaglandin infusion to maintain ductal patency and should be transferred promptly to pediatric cardiac surgery centers. With timely intervention and in the absence of additional complications, most newborns can achieve excellent postoperative recovery. Also multicenter, large-scale prospective studies with extended follow-up are required to more accurately assess long-term prognosis in affected newborns.

Authors' contributions

Junshu Xie and Fufen Yin summarized information and wrote the main manuscript text. Xiuju Yin and Tiantian Han provided obstetric patient information. Qiuyan Pei provided ultrasound information. Lin Zhang provided prenatal diagnostic information. Huili Zhang and Shoujun Li provided information on neonatology pediatric cardiac surgery data. Xiaohong Zhang designed research plan and guided the writing of the manuscript. All authors reviewed the manuscript.

Funding

No funding.

Data availability

The datasets used and/or analysed during the current study are available from the corresponding author on reasonable request.

Declarations

Ethics approval and consent to participate

This study was approved by the Ethics Committee of Peking University People’s Hospital (Approval No.: 2025PHB407-001), and all pregnant women signed informed consent forms. And our study adhered to the Declaration of Helsinki.

All authors consent to publish this manuscript.

Consent for publication

All authors have consent and agree to publish this manuscript.

Competing interests

The authors declare no competing interests.