The role of cardiac surgeons in online prenatal counselling for congenital heart disease.

Introduction

Congenital heart disease (CHD) is the most common birth defect in China and a main cause of death and disability in newborns and infants.[1] Technological developments have led to continuous improvements in ultrasound diagnostic techniques for foetal heart examination and now many CHDs can be detected and diagnosed during the foetal period. Prenatal counselling for parents of foetuses with abnormal cardiovascular function detected by ultrasound can reduce the birth incidence of severe CHD, reduce infant mortality and improve population health.[2,3] Paediatric cardiovascular specialists can evaluate the prognosis and outcome of foetuses with abnormal cardiovascular systems using foetal echocardiography, and can provide professional advice and guidance for family members.[4] However, at present, medical resources are limited in China. Most primary hospitals or maternal and child health centres lack professional cardiac surgeons, which makes it difficult for many families of foetuses with cardiovascular abnormalities to obtain professional prenatal counselling, and leads to adverse pregnancy outcomes. The Good Doctor Online and WeChat platforms are common communication platforms for doctors and patients; because they are not limited by time and space, they provide a convenient channel for doctor–patient communication. The Good Doctor Online was established in 2006 and is a leading Internet medical platform in China. It has achieved a leading position in the fields of graphic consultation, telephone consultation, telemedicine services and post-treatment management. Doctors can provide direct medical consultations, popular science information, information about disease management, appointments and other services to patients through Good Doctor Online, helping patients to obtain expert advice without time and space constraints. WeChat is a tool that has become widely used by the public in recent years. Using this platform, doctors can communicate with patients through information, pictures, text, video and verbal messaging. Patients can consult experts without time and space restrictions. WeChat provides good consulting conditions for patients in remote areas who lack medical resources. Through the Good Doctor Online and the WeChat platforms, the author receives a large number of prenatal counselling requests for family members of foetuses with cardiovascular abnormalities every year, provides professional prenatal counselling for them, carries out a close follow-up of foetuses after birth, and formulates postnatal treatment plans. Good results have been obtained using this method. This paper summarizes 400 cases of foetuses with cardiovascular abnormalities for which the author has provided consultations and evaluates this online counselling method.

Methods

Data

A total of 400 cases of prenatal consultation for foetal cardiovascular abnormalities using the Good Doctor Online and WeChat platforms from January 2014 to December 2017 were selected. Age, gestational weeks, parity and foetal cardiovascular abnormalities were recorded. Foetal ultrasound reports were all from local hospitals. If the ultrasound results were unsatisfactory, it was recommended that foetal ultrasound examinations should be obtained from local qualified hospitals. For foetal cardiovascular abnormalities detected before 28 weeks, a second consultation in 2 weeks after re-examination in a qualified hospital was recommended. Ethical approval was obtained from the ethics committee of Beijing Children’s Hospital, Capital Medical University, National Center for Children’s Health. All patients provided written informed consent.

Consultation for foetal cardiovascular anomalies

The pathology of foetal cardiovascular anomalies includes intracardiac bright spot, ventricular septal defect, atrial septal defect, single ventricle, single atrium, absence of ductus arteriosus, acute atrioventricular valve, acute arterial valve, abnormal pulmonary vein, abnormal aortic arch, abnormal main artery, abnormal ventricular wall, cardiac tumour, abnormal cardiac rhythm and pericardial effusion. Owing to limitations in the sound transmission window in the foetal period, it is difficult to diagnose some cardiovascular malformations before delivery, which may lead to missed diagnoses. Such malformations may only be diagnosed after birth. Umbilical cord blood tests or amniocentesis are recommended to analyse foetal chromosomes in pregnant women over 30 years old, in women whose first child was diagnosed with CHD and in those with a history of stillbirth or unexplained abortion.

Pregnancy choice

Through the Good Doctor Online and WeChat platforms, the author can obtain detailed information about pregnant women’s personal history, family history, birth history and type of pregnancy, including whether the pregnancy is characterized by other organ malformations or chromosomal abnormalities. The author explains factors such as the abnormalities of the foetal cardiovascular system, possible future development, the stage of the operation, complications, long-term prognosis and the estimated cost of surgery. Suggestions for termination of pregnancy, possible continuation of pregnancy and continuation of pregnancy are made according to the specific foetal cardiovascular system abnormalities, and whether there are other simultaneous organ malformations and chromosomal abnormalities. Pregnant women and their families make the decision whether to continue the pregnancy or not.

Follow-up of foetuses with cardiovascular abnormalities

For pregnant women who continue pregnancy, timely follow-up through the Good Doctor Online or WeChat platforms is conducted to monitor the health status of newborns after birth. For conditions such as cardiac tumours, pericardial effusion and intracardial bright spot, it is recommended that the foetus is re-examined 3 to 12 months after birth; for other types of foetal cardiovascular abnormalities, it is recommended that the foetus is examined immediately after birth using echocardiography or cardiovascular computerized tomography scan, and corresponding treatment measures are formulated. Follow-up and interventions for foetal and neonatal cardiovascular abnormalities are registered and followed up until the child is 1 year old.

Statistical methods

SPSS 20.0 software (IBM Corp., Armonk, NY, USA) was used, and frequency data were expressed as percentages, means ± standard deviations and ranges. Chi-squared tests were used to compare rates; odds ratios and/or risk ratios with 95% confidence intervals were used for analysis. P < 0.05 was considered to indicate statistical significance.

Results

Demographic data

There were 400 cases of pregnant women aged 25.74 ± 2.87 years (18–41 years). Foetal cardiovascular abnormalities were detected at 26.84 ± 4.31 weeks (22–38 weeks). Of these cardiovascular abnormality cases, 334 were at 22 to 27 ± 6 weeks and 66 at 28 to 38 weeks. Regarding parity, 305 cases were the first foetus, 78 the second foetus and 17 the third foetus. There were 28 foetal chromosome examinations, of which 2 showed trisomy 21 syndrome and 26 were normal.

Foetal cardiovascular abnormalities detected

Foetal cardiovascular abnormalities comprised 248 simple cardiac abnormalities and 152 complex cardiac abnormalities. Of the 248 simple cardiac abnormality cases, the most frequent abnormalities were intracardiac bright spot (64 cases), double aortic arch and vascular ring (57), ventricular septal defect (44), and mild mitral or tricuspid regurgitation (25). Of the 152 cases of complex cardiac abnormalities, the most frequent abnormalities were tetralogy of Fallot (56 cases), aortic arch coarctation or interruption (24), complete atrioventricular septal defect (12), and severe mitral or tricuspid stenosis or atresia (11) (see Table 1).

Table 1.

Cardiovascular abnormalities detected by foetal echocardiography.

Type	No. of cases	Consultant suggestion	Family members decide to induce/continue pregnancy	Intrauterine deaths	No. of births	No. of neonatal deaths	No. of operative cases
Simple type	248	Pregnancy can be continued	2/246	0	246	0	9
Intracardiac bright spot	64	Pregnancy can be continued	0/64	0	64	0	0
Ventricular septal defect	44	Pregnancy can be continued	1/43	0	43	0	9
Double aortic arch and vascular ring	57	Pregnancy can be continued	0/57	0	57	0	0
Mitral or tricuspid regurgitation	35	Pregnancy can be continued	0/35	0	35	0	0
Aberrant right subclavian artery	21	Pregnancy can be continued	0/21	0	21	0	0
Persistent left superior vena cava	19	Pregnancy can be continued	0/19	0	19	0	0
Pericardial effusion	4	Pregnancy can be continued	0/4	0	4	0	0
Arrhythmia	3	Pregnancy can be continued	0/3	0	3	0	0
Cardiac neoplasm	1	Pregnancy can be continued	1/0	0	0	0	–
Complex type	152		126/26	4	22	4	18
Tetralogy of Fallot	56	Possible pregnancy	42/14	3	11	1	10
Aortic arch coarctation or interruption	24	Possible pregnancy	18/6	1	5	2	3
Complete atrioventricular septal defect	12	Possible pregnancy	10/2	0	2	0	2
Severe mitral or tricuspid valve stenosis or atresia	11	Possible pregnancy	11/0	0	0	0	0
Severe pulmonary artery stenosis or atresia	10	Possible pregnancy	10/0	0	0	0	0
Right ventricle double outlet	9	Possible pregnancy	8/1	0	1	0	1
Complete macrovascular malposition	8	Pregnancy termination	6/2	0	2	1	1
Left ventricular dysplasia	6	Pregnancy termination	6/0	0	0	0	0
Single ventricle	5	Pregnancy termination	5/0	0	0	0	0
Persistent trunk arteries	3	Possible pregnancy	3/0	0	0	0	0
Tricuspid valve descending malformation	2	Possible pregnancy	2/0	0	0	0	0
Severe aortic stenosis or atresia	2	Possible pregnancy	2/0	0	0	0	0
Single atrium	1	Possible pregnancy	1/0	0	0	0	0
Right pulmonary artery originating from aorta	1	Possible pregnancy	0/1	0	1	0	1
Absent ductus arteriosus	1	Possible pregnancy	1/0	0	0	0	0
Complete anomalous pulmonary venous drainage	1	Possible pregnancy	1/0	0	0	0	0
Total	400		128/272	4	262	4	27

Pregnancy outcome

Two (0.8%) of the 248 foetuses with simple cardiac abnormalities and 103 (67.8%) of the 152 foetuses with complex cardiac abnormalities had induced abortion. The foetal induction rate for complex cardiac abnormalities was higher than for simple cardiac abnormalities (P < 0.05) (see Table 2).

Table 2.

Comparison of simple and complex foetal cardiac abnormalities in induced abortion and continued pregnancy.

Pregnancy outcome	Number of cases	Simple type (n = 248)	Complex type (n = 152)
Induced abortion		2 (0.8%)	126 (67.8%)
Continued pregnancy		246 (99.2%)	26 (32.2%)
Χ2 value		218.239
P value		<0.001
Odds ratio		2.25

Postnatal outcome of foetuses with cardiac abnormalities detected by echocardiography

Of the 248 foetuses with simple cardiac abnormalities, trisomy 21 syndrome was detected in one case of ventricular septal defect after induced labour; 7 of the remaining 43 cases closed naturally during the foetal period, 9 cases underwent ventricular septal defect repair within 6 months after birth and recovered well after surgery and the other 27 cases were followed up. After birth, ultrasound was normal and no clinical symptoms were found in patients with double aortic arch and vascular ring. Some cases of mild mitral or tricuspid regurgitation persisted and some disappeared; ultrasound showed that the cardiac cavity was normal and the persistent cases continue to be followed up. Patients with aberrant right subclavian artery were normal and had no clinical symptoms. Conditions of persistent left superior vena cava persisted, but did not affect cardiac function. The pericardial effusion disappeared within 6 months after birth. The arrhythmia changed to a normal sinus rhythm within 6 months after birth. After some consideration, the family members of the foetus with a cardiac tumour requested an induced abortion.

Of the 152 foetuses with complex cardiac abnormalities, mothers of 103 chose induced abortion and 26 chose to continue pregnancy (including 14 cases of tetralogy of Fallot, 6 cases of coarctation or interruption of aortic arch, 2 cases of complete atrioventricular septal defect, 1 case of double outlet of right ventricle, 2 cases of complete macrovascular malposition and 1 case of right pulmonary artery originating from aorta). Of the 26 cases of continued pregnancy, there were 4 intrauterine deaths and 22 births. Ultrasound examination was performed immediately after birth. The child’s condition was assessed by a cardiac surgeon and an operative plan was formulated. Of 14 cases of tetralogy of Fallot, 3 died in uterus, 11 were born, 1 died awaiting surgery and 10 received surgery in time. Of the 6 cases of coarctation or interruption of aortic arch, 1 died in uterus, 2 died after birth and 3 received surgery in time. Of the 2 cases of complete macrovascular malposition, 1 died after birth and 1 case received surgery in time. All of the 2 cases of atrioventricle septal defect, 1 case of double outlet of right ventricle and 1 case of origin of right pulmonary artery originating from aorta received surgery in time (see Table 1).

Discussion

CHD is very common and is the most serious birth defect. The mortality and disability rate from severe CHD are relatively high.[5] With continuous developments in paediatric cardiovascular medicine and cardiovascular surgery, most CHDs can be cured by interventional therapy or surgical treatment after birth, but many CHDs are diagnosed after birth. At the time of consultation, the children already have heart failure; although some may survive infancy, they may experience irreversible heart and lung changes and the chance of radical cure is lower for serious cases.[6]

As ultrasound technology develops, the diagnosis of CHD has advanced to the foetal stage. Foetal diagnosis of CHD can avoid unnecessary induced abortion of foetuses with simple CHD and the birth of foetuses with complex CHD and poor prognosis.[7] In addition, foetal diagnosis with good prognosis and viable treatment options can prompt decisions to continue pregnancy. Foetal diagnosis extends CHD treatment plans to the prenatal stage.[8,9] Most CHDs can be diagnosed using ultrasonography. The choice of foetal induced abortion or continued pregnancy should be based on the type of CHD, the feelings of pregnant women and their families, the treatment level of the CHD and other factors. The choices made by pregnant women and their families are mainly based on medical information provided by doctors.[10] At present, once the foetus has been diagnosed with CHD, most women are induced to give birth. Therefore, professional and detailed prenatal counselling for pregnant women and their families during pregnancy is of great value.

Prenatal counselling needs to help pregnant women and their families make choices according to their personal history, birth history, family history and mode of conception, the economic and psychological consequences of having a baby with CHD, the possible future development of the infant and the level of medical intervention available.[3,11,12] Because CHD is often accompanied by other systemic malformations, it is recommended that foetuses with CHD undergo umbilical cord blood examination or amniocentesis, and the retention of foetuses should be considered after excluding multiple malformations and hereditary diseases.[13] Prenatal consultation for CHD requires a doctor with extensive knowledge of prevention, diagnosis and treatment of CHD. Obstetricians or neonatologists have relatively little experience of CHD pregnancies. Therefore, such cases are best managed by paediatric cardiologists or cardiac surgeons.[14-16]

In China, there are insufficient paediatric cardiac surgeons and resources are unevenly distributed. Most primary hospitals lack both suitable technology and doctors with experience in preventing, diagnosing and treating CHD. In rural or remote areas, prenatal examination is not well understood, and diagnosis, treatment and prognosis for foetal cardiovascular abnormalities are insufficient. Lack of understanding can result in induced abortion for a large number of foetuses with simple CHD and good prognosis, and the birth rate of foetuses with complex CHD with poor prognosis and no effective treatment measures has increased. Prenatal consultations on the Good Doctor Online and WeChat platforms can effectively reduce the induced abortion rate of foetuses with simple CHD and good prognosis, and reduce the birth rate of foetuses with complex CHD and poor prognosis. For the retained CHD foetuses, close follow-ups are conducted and postnatal treatment programs formulated, so that most of these foetuses receive timely treatment after birth.

In conclusion, prenatal consultations by cardiac surgeons using the Good Doctor Online and WeChat platforms can reduce the induced abortion rate of foetuses with simple CHD and reduce the birth rate of foetuses with complex CHD. This method can also effectively improve the prognosis of children with CHD.