Skip to main content

Incidence, prognosis, and perinatal outcomes of and risk factors for severe twin–twin transfusion syndrome with right ventricular outflow tract obstruction in the recipient twin after fetoscopic laser photocoagulation

Abstract

Background

Right ventricular outflow tract obstruction (RVOTO) is the most frequently encountered congenital heart disease in patients with twin –twin transfusion syndrome (TTTS) and is especially prevalent in the recipient twin. In this retrospective study, we evaluated the incidence, prognosis, postnatal management, and perinatal outcomes of and risk factors for RVOTO in the recipient twin in severe TTTS cases which diagnosed before 26 weeks after fetoscopic laser photocoagulation (FLP) at a single center in Taiwan.

Methods

RVOTO was diagnosed using fetal or postnatal echocardiography. The fetal outcomes evaluated were perinatal survival rate, neonatal brain image anomalies rate, gestational age at delivery, and birth weight.

Results

Total 187 severe TTTS cases were included; 14 (7.49%) had a recipient twin with RVOTO (12 cases of pulmonary stenosis and 2 of pulmonary atresia). Of these 14 cases, 3 (21.4%) demonstrated improvements in outflow obstruction after FLP, and 11 (78.6%) resulted in perinatal survival. Of the 11 survivors, 5 (45.5%) received transcatheter balloon valvuloplasty to alleviate the RVOTO. The perinatal survival rate, gestational age at delivery, neonatal brain image anomaly rate, and birth weights did not significantly differ between the groups in which the recipient twin had versus did not have RVOTO. Generally, the recipient twin had RVOTO received FLP at a younger gestational age (in weeks; 19.3 ± 2.4 vs. 20.7 ± 2.6, p = 0.048) and had a higher percentage of cases at Quintero stage IV (50.0% vs. 12.1%, p < 0.001) than those in which the recipient twin did not have with RVOTO. Using logistic regression, we discovered that FLP at a younger gestational age (p = 0.046, odds ratio = 0.779) and TTTS at Quintero stage IV (p = 0.001, odds ratio = 7.206) were risk factors for the recipient twin developing RVOTO after FLP in severe TTTS cases.

Conclusions

The post-FLP perinatal outcomes of cases of severe TTTS in which the recipient twin had versus did not have RVOTO were comparable in this study, which may have been due to the similar gestational ages at delivery and strong influence of high Quintero stages (stages III and IV).

Peer Review reports

Background

Twin–twin transfusion syndrome (TTTS) is a complication that occurs in approximately 9% of monochorionic diamniotic twin pregnancies [1] due to unbalanced intertwin placenta flow with preferential shunting of blood from one twin (donor) to the other (recipient) through vascular communication. As discussed by Quintero et al. [2], TTTS is clinically characterized by concurrent polyhydramnios in the “recipient” twin and oligohydramnios in the “donor” twin, as identified through ultrasound. Generally, the donor twin exhibits hypovolemia, and the recipient exhibits hypervolemia. To compensate for the hypovolemia, the donor twin secretes vasoactive mediators, such as endothelin I and renin, which circulate to the recipient twin through intertwin anastomoses; the vasoactive substances can cause polyhydramnios, cardiomyopathy, and atrioventricular valve regurgitation in the recipient twin [3,4,5]. In 70% of TTTS cases, the recipient twin demonstrates echocardiographic signs of cardiovascular complications at the time of diagnosis [6, 7].

In TTTS survivors, the incidence of congenital heart disease can be as high as 87 per 1000 live births, a 12-fold increased incidence compared with that for singletons [8]. However, the mechanism underlying congenital heart disease development in cases of TTTS is not completely understood; multiple factors have been proposed, such as hemodynamic derangements in the donor and recipient twins, genetic predisposition in twinning, and abnormal placentation [9]. Right ventricular outlet obstruction (RVOTO), such as in pulmonary stenosis (PS) and pulmonary atresia (PA), is the most prevalent form of congenital heart disease, with a reported incidence of 6.7%–12.9% in recipient twins [10,11,12,13]. This higher risk exists because recipient twins have high risks of right heart dysfunction, cardiomegaly, and tricuspid valve regurgitation, which can cause the flow through the pulmonary valve to decrease, potentially leading to narrowing and stenosis of the fetal pulmonary valve of the recipient twin [6]. The etiology of the higher incidence of cardiomyopathy and the lower pulmonary valve flow in the recipient twin compared with the donor twin has been hypothesized as being the result of differing levels of renin–angiotensin activity between the recipient and donor twins [14]. The recipient twin may have higher renin–angiotensin activity than the donor twin, leading higher incidence of RVOTO in recipient twins [15]. Differences in volume overload, with volume overload occurring more frequently in recipient than in donor twins due to intermittent absent or reversed umbilical artery end-diastolic flow, have also been reported to increase the incidence of recipient-twin RVOTO [16]. However, the true etiology of RVOTO in recipient twins remains unclear [17].

Fetoscopic laser photocoagulation (FLP) is the first-line therapy for TTTS diagnosed prior to 26 weeks of gestation [18,19,20]. Because FLP can interrupt intertwin anastomoses, it blocks the vasoactive mediators from the donor to the recipient twin. Although regression has been achieved following FLP in many cases of RVOTO in recipient twins [17, 21, 22], the incidence of RVOTO in recipient twins after FLP remains high [8]. Furthermore, persistent RVOTO in recipient twins with TTTS after FLP has been reported to be associated with decreased survival of fetuses [11, 17] and a less favorable neonatal outcome in cases of PA [10]. Furthermore, a study demonstrated that RVOTO in recipient twins has no effect on donor twin survival [13].

Our institution is the largest laser center for treatment of TTTS cases in Taiwan. In this study, we included cases of severe TTTS ( those diagnosed before 26 weeks of gestation) to analyze the incidence, prognosis, and postnatal management of and the risk factors for RVOTO in recipient twins and the influence of RVOTO in recipient twins on perinatal outcomes, including survival rate, gestational age of delivery, birth weight, and neonatal brain image results.

Methods

This retrospective study was performed at the Chang Gung Memorial Hospital of Taoyuan, Taiwan, and it was approved by the Institutional Review Board of the Chang Gung Medical Foundation (202101274B0). The diagnosis of TTTS was based on ultrasound findings, as defined by Quintero et al. [2]. We included TTTS cases from October 2007 to March 2021 diagnosed before the gestational age of 26 weeks and that were treated with FLP at our hospital. The FLP procedure for TTTS has been described previously [23].

A complete evaluation of the fetal structure was conducted with a Voluson Expert 8 (GE Medical Systems, Milwaukee, WI, USA) to assess the fetal cardiac anatomy in all patients with TTTS before FLP. Color and pulsed-wave Doppler measurements were obtained in the absence of fetal breathing and movements. All cases of suspected PS or PA at the diagnosis of TTTS were referred for fetal echocardiography performed by a perinatal cardiologist. The diagnosis of PS was based on high peak systolic velocity (>90 cm/s) and turbulent flow across the pulmonary valve, with or without thickened and domed pulmonary valve cusps, which are associated with structural or functional right heart abnormalities. PA was diagnosed in the absence of forward flow across the pulmonary valve during systolic and reverse flow in the ductus arteriosus [24]. Cases associated with major cardiac anomalies other than PS and PA, such as Ebstein’s anomaly and tetralogy of fallot (TOF), or genetic anomalies, such as trisomy, were excluded from this study. Patients with TTTS were followed up with serial ultrasound 1 day before and every day after laser therapy if the patient was admitted. In cases of ultrasound-based PS or PA diagnosis, the condition of the fetal cardiac structure of the recipient twins was followed up in the outpatient department at every visit.

After a delivery in which the recipient twin survived, the pulmonary valve pathology was confirmed in the recipient twin through postnatal echocardiography or angiographic examinations if transcatheter balloon valvuloplasty (TBV) was performed. Perinatal survival was defined as survival 28 days after delivery.

Because fetal survival and intact neurological status are key outcomes for fetuses with TTTS after FLP, all living neonates with TTTS who had undergone FLP received cranial ultrasound examinations within 1 month of delivery. If two or more cranial ultrasound tests were performed, the most recent result was used. Mild cerebral image anomalies were defined as the presence of at least one of the following: intraventricular hemorrhage (IVH) of grade I or II; lenticulostriate vasculopathy; and subependymal pseudocysts. Severe cerebral image anomalies were defined as the presence of at least one of the following: IVH of grade III or IV; cystic periventricular leukomalacia of grade II or higher; porencephalic cysts; and ventricular dilatation. Ventricular dilatation was considered present when the width of the unilateral or both lateral ventricles exceeded that in the 97th percentile [23].

Statistical analysis was conducted using SPSS (version 11.0 for Windows; SPSS, Chicago, IL, USA). The normality of the data was assessed using the Shapiro–Wilk test. The Student’s t test or Mann–Whitney U test was used to compare continuous variables between groups. Qualitative data were compared using the χ2 test or Fisher’s exact test. A p value of less than 0.05 was considered significant. Logistic regression (forward condition) was used to determine the RVOTO risk factors in recipient twins. The gestational age at FLP, Quintero stage, maternal age at operation, and maximum vertical pocket of amniotic fluid (MVP) were included in to the mode when the p value for the variable was less than 0.05, and the variables were removed when the p value was greater than 0.1. The odds ratios of the variables are expressed as the odds (95% confidence interval).

Results

After exclusion of two cases with selective termination after laser therapy due to discordant fetal major brain image anomaly in one fetus, two cases of self-termination due to personal factors, one case of Ebstein’s anomaly in the recipient twin, one case of trisomy 21 detected after FLP, one case of donor TOF, and two cases lost to follow-up, a total of 187 cases of severe TTTS receiving FLP were included (Figure 1). The preoperational characteristics and fetal survival rates after FLP are listed in Table 1. RVOTO was identified in the recipient twin in 14 (7.49%) of the 187 TTTS cases. Maternal age at operation, Quintero stage, interval between operation and delivery, and fetal survival did not significantly differ between the cases of TTTS with versus without recipient twins with RVOTO. The gestational age upon FLP was lower among the TTTS cases in which the recipient twin had RVOTO. Although the Quintero stage distributions of the two groups were significantly different, the percentage of cases with a high Quintero stage (stage III or IV) did not significantly differ. Perinatal survival was achieved in 11 (78.6%) of the 14 cases with recipient twins with RVOTO and in 130 (75.1%) of the 173 cases without recipient twins with RVOTO, indicating a comparable rate of perinatal survival between the two groups. Three (21.4%) of the 14 cases with recipient twins with RVOTO demonstrated improvements after FLP, and in five cases (45.5%), TBV was required to alleviate the RVOTO.

Fig. 1
figure 1

The flowchart of severe TTTS received FLP included and excluded into this study. TTTS: twin-twin transfusion syndrome. FLP: fetoscopic laser photocoagulation. TOF: tetralogy of Fallot

Table 1 Characteristics of the patients with twin-to-twin transfusion syndrome patients with and without recipient right ventricular obstruction (RVOTO) before fetoscopic laser therapy

The birth weights of the recipient and donor twins and the results of the fetal brain imaging in the cases of severe TTTS after FLP with recipient twin perinatal survival are listed in Table 2. No significant difference was identified in the birth weights of the recipient and donor twins or in the neonatal image anomaly rates of the TTTS groups with and without recipient twins with RVOTO. The neonatal brain image anomaly rate was 3.71% (10/268) in the 141 TTTS cases with recipient twin survival (268 total surviving fetuses) after FLP, and the severe brain image anomaly rate in the 141 TTTS cases was 0.74% (2/268; Table 2).

Table 2 Characteristics of the patients with severe twin-to-twin transfusion syndrome with and without recipient right ventricular obstruction (RVOTO) post fetoscopic laser therapy with recipient survival

The individual diagnoses and brief histories of the 14 patients with severe TTTS with recipient twins with RVOTO are listed in Table 3. One patient did not receive a formal diagnosis of PS through fetal echocardiography before FLP (case 14); mild PS was identified after delivery through neonatal echocardiography.

Table 3 List of severe twin-twin transfusion syndrome with recipient twin right ventricular outflow tract obstruction

To identify the risk factors for recipient twins developing RVOTO, logistic regression was employed, with maternal age and gestational age at FLP, high Quintero stage, Quintero stage IV, and MVP of the recipient twin used as variables. Young gestational age at FLP and Quintero stage IV were identified as risk factors for a recipient twin developing RVOTO in cases of severe TTTS (Table 4).

Table 4 Predisposing factors for recipient RVOTO in severe TTTS cases

Discussion

The results of this study revealed that the prevalence of RVOTO in recipient twins affected by severe TTTS was 7.49%, and RVOTO was improved in 21.4% (3/14) of recipient twins after FLP. Furthermore, the incidence of RVOTO in recipient twins with perinatal survival after FLP was 7.8% (11/141), and 45.5% (5/11) of the surviving recipient twins with RVOTO required TBV to alleviate outflow obstruction symptoms. The post-FLP prognoses of severe TTTS in cases of a recipient twin with versus without RVOTO were comparable, and the two groups had similar neonatal survival rates, gestational ages at delivery, birth weights, and brain image anomaly rates.

The prevalence of congenital heart disease in the general population is approximately 1% [25], and the prevalence of RVOTO ranges from 0.05% to 0.15% [26]. In our study, the incidence of RVOTO in recipient twins for severe TTTS cases was 7.49% before FLP and 7.8% after delivery, much higher incidence than that in the general public. These findings indicate that fetal echocardiography should be performed for fetuses with TTTS to identify any cardiac anomalies. However, because the post-FLP outcomes of TTTS in which the recipient twin has RVOTO are comparable with those of TTTS in which the recipient twin does not have RVOTO and because RVOTO can improve after FLP, a prenatal diagnosis of RVOTO in a TTTS recipient twin is probably not a poor prognostic factor and is not an indicator that FLP should not be administered. FLP was reported to improve the pulmonary valve flow in recipient twins; this is probably because FLP improves right ventricular systolic and diastolic functions [27]. However, the prevalence of TTTS with congenital heart disease at birth may be influenced by the survival rate of patients with TTTS being improved after treatment with FLP [8, 28]. FLP may not reduce the incidence of RVOTO in surviving recipient twins because the survival rate of recipient twins both with and without RVOTO generally improves with application of FLP. Moreover, the pediatric cardiac echography–detected 7.8% (11/141) incidence of RVOTO after FLP in the surviving recipient twins of our study further emphasizes the importance of pediatric cardiac echography for survivors of TTTS.

The prevalence of prenatal RVOTO occurring only after laser therapy was reported as 5.6% [10]. As was true of our case 14 (Table 3), in such cases RVOTO can only be identified after delivery, and therefore, RVOTO may develop after laser therapy. We generally check the fetal color Doppler echocardiograms of fetuses with TTTS before and after FLP. However, in clinical follow-ups after FLP, two-dimensional echocardiography is performed only in cases without prelaser echocardiography and immediately after laser RVOTO diagnosis. Therefore, mild PS that did not develop immediately after FLP may be overlooked. Moreover, if mild PS develops after FLP and subsides before delivery, we may not discover the PS through pediatric cardiography. Consequently, the incidence of RVOTO in recipient twins after FLP may be higher than we report.

Onset of TTTS early in gestation was reported as a risk factor for RVOTO in TTTS recipient twins, which may have been due to increased vulnerability to hemodynamic imbalances in the fetal heart in early pregnancy [29]. We also discovered that recipient twins developed RVOTO more often when TTTS was diagnosed as Quintero stage IV and FLP was performed at younger gestational age. In our institution, we generally treat cases of TTTS within 1 or 2 days of diagnosis. Therefore, in our institution, young gestational age upon FLP also indicates young gestational age at diagnosis. Our data revealed the prevalence of recipient RVOTO to be highest in cases with Quintero stage IV, which supports the findings of a report also demonstrating that the rate of recipient twins developing RVOTO was higher in cases of Quintero stage III–IV TTTS [11]. However, research also indicated that the risk of developing RVOTO does not differ in any Quintero stage [12]. Recipient twins in cases of stage IV TTTS are more likely to develop cardiomyopathy with right ventricle (RV) dilation and hypertrophy. The tricuspid valve regurgitation and aortic blood flow reversal through the ductus arteriosus in TTTS can cause the pulmonary valve flow to decrease, which can lead to narrowing and stenosis of the fetal pulmonary valve in the recipient twin. This may explain why stage IV TTTS is a risk factor for RVOTO. However, because cardiomyopathy occurs less often in TTTS donor twins, RVOTO has been reported less often in donor twins.

TTTS in which the recipient twin has RVOTO after laser therapy was documented as being associated with poorer outcomes [11]. In addition, high Quintero stage TTTS (stage III or IV) has been linked to poorer outcomes after FLP [30, 31], and 66.7%– 92.5% of TTTS cases in which the recipient twin has RVOTO have a high Quintero stage [11, 13, 29]. In our study, the percentages of high Quintero stage TTTS in the groups with and without recipient twins with RVOTO were similar (57.1% vs. 49.7%, respectively, p = 0.78); this similarity may explain why the TTTS group with recipient twins with RVOTO had comparable outcomes, including survival and normal cranial image rates, to those of the TTTS group with recipient twins without RVOTO after FLP. Through this study, we discovered that TTTS in which the recipient twin has RVOTO was not associated with higher incidence of neonatal ultrasound brain image anomalies. In our previous study, we demonstrated that the key predisposing factor for severe brain imaging anomalies in survivors of TTTS after FLP was younger gestational age at delivery [32]. Another report stressed that survivors with older gestational age at the time of FLP, younger gestational age at delivery, and lower birth weight have higher risk of developing neurodevelopmental impairment [33]. According to our data, the gestational age at delivery and birth weights of the donor and recipient twins did not significantly differ between the TTTS groups in which the recipient twin had versus did not have RVOTO (Table 1). This may explain the similarity in the brain image anomaly rates of the two TTTS groups.

RVOTO mainly comprises PA and PS; in our series, the incidence of PA in recipient twins with RVOTO was 14.3% (2/14). However, other studies have reported the risk of PA to be 28.5% (2/7) [10], 45% (24/53) [17], 19.2% (5/26) [9], and 42.8% (12/28) [11]. Therefore, the incidence of PA in recipient twins with RVOTO was relatively low in our study. PA has a poorer prognosis than PS does [34]; this may have contributed to the comparable outcomes of the TTTS groups with recipient twins with versus without RVOTO after FLP in our study.

The strength of this study is that it was based on data from a single center with consecutive cases. Operations were performed following the same surgical indications, and similar procedures and instruments were employed in all of the cases. The limitations of this study include the small number of cases, the low percentage of PA cases, and the possibility that the neonatal brain ultrasound results may not have revealed all cerebral injuries in the included neonates [32].

Conclusions

In this study, the incidence of RVOTO in the recipient twins of cases of severe TTTS was 7.49%; Quintero stage IV and young gestational age at FLP were two risk factors for the recipient twin developing RVOTO in severe cases of TTTS. The perinatal outcomes (gestational age at delivery, perinatal survival rate, birth weight, and neonatal brain image anomaly rate) of the TTTS groups in which the recipient twin had versus did not have RVOTO after FLP did not significantly differ. This may have been because of the similar percentage of high Quintero stage in the two groups and the relative low percentage of PA in TTTS cases in which the recipient twin had RVOTO.

Availability of data and materials

The datasets obtained and analyzed in this study are available from the corresponding author upon reasonable request.

Abbreviations

RVOTO:

Right ventricle outlet obstruction

TTTS:

Twin–twin transfusion syndrome

FLP:

Fetoscopic laser photocoagulation

PS:

Pulmonary stenosis

PA:

Pulmonary atresia

TBV:

Transcatheter balloon valvuloplasty

MVP:

Maximum vertical pocket

IVH:

Intraventricular hemorrhage

PVL:

Periventricular leukomalacia

TOF:

Tetralogy of fallot

References

  1. Lewi L, Jani J, Blickstein I, Huber A, Gucciardo L, Van Mieghem T, et al. The outcome of monochorionic diamniotic twin gestations in the era of invasive fetal therapy: a prospective cohort study. Am J Obstet Gynecol. 2008;199(5):514 e511–8.

    Google Scholar 

  2. Quintero RA, Morales WJ, Allen MH, Bornick PW, Johnson PK, Kruger M. Staging of twin-twin transfusion syndrome. J Perinatol. 1999;19(8 Pt 1):550–5.

    Article  CAS  PubMed  Google Scholar 

  3. Marton T, Hajdu J, Papp C, Patkos P, Hruby E, Papp Z. Pulmonary stenosis and reactive right ventricular hypertrophy in the recipient fetus as a consequence of twin-to-twin transfusion. Prenat Diagn. 2001;21(6):452–6.

    Article  CAS  PubMed  Google Scholar 

  4. Barrea C, Alkazaleh F, Ryan G, McCrindle BW, Roberts A, Bigras JL, et al. Prenatal cardiovascular manifestations in the twin-to-twin transfusion syndrome recipients and the impact of therapeutic amnioreduction. Am J Obstet Gynecol. 2005;192(3):892–902.

    Article  PubMed  Google Scholar 

  5. Michelfelder E, Gottliebson W, Border W, Kinsel M, Polzin W, Livingston J, et al. Early manifestations and spectrum of recipient twin cardiomyopathy in twin-twin transfusion syndrome: relation to Quintero stage. Ultrasound Obstet Gynecol. 2007;30(7):965–71.

    Article  CAS  PubMed  Google Scholar 

  6. Rotar IC, Zaharie G, Staicu A, Preda A, Muresan D. Fetal cardiovascular alterations in twin-to-twin transfusion syndrome. Med Pharm Rep. 2020;93(1):5–11.

    PubMed  PubMed Central  Google Scholar 

  7. Karatza AA, Wolfenden JL, Taylor MJ, Wee L, Fisk NM, Gardiner HM. Influence of twin-twin transfusion syndrome on fetal cardiovascular structure and function: prospective case-control study of 136 monochorionic twin pregnancies. Heart. 2002;88(3):271–7.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  8. Gijtenbeek M, Shirzada MR, Ten Harkel ADJ, Oepkes D, M CH. Congenital Heart Defects in Monochorionic Twins: a systematic review and meta-analysis. J Clin Med. 2019:8(6):902.

  9. Pruetz JD, Votava-Smith JK, Chmait HR, Korst LM, Llanes A, Chmait RH. Recipient twin circular shunt physiology before fetal laser surgery: survival and risks for postnatal right ventricular outflow tract obstruction. J Ultrasound Med. 2017;36(8):1595–605.

    Article  PubMed  Google Scholar 

  10. Lougheed J, Sinclair BG, Fung Kee Fung K, Bigras JL, Ryan G, Smallhorn JF, et al. Acquired right ventricular outflow tract obstruction in the recipient twin in twin-twin transfusion syndrome. J Am Coll Cardiol. 2001;38(5):1533–8.

    Article  CAS  PubMed  Google Scholar 

  11. Ortiz JU, Masoller N, Gomez O, Bennasar M, Eixarch E, Lobmaier SM, et al. Rate and outcomes of pulmonary stenosis and functional pulmonary atresia in recipient twins with twin-twin transfusion syndrome. Fetal Diagn Ther. 2017;41(3):191–6.

    Article  PubMed  Google Scholar 

  12. Eschbach SJ, Ten Harkel ADJ, Middeldorp JM, Klumper F, Oepkes D, Lopriore E, et al. Acquired right ventricular outflow tract obstruction in twin-to-twin transfusion syndrome; a prospective longitudinal study. Prenat Diagn. 2018;38(13):1013–9.

    Article  PubMed  Google Scholar 

  13. Murata S, Nakata M, Sugino N. Prevalence of right ventricular outflow tract abnormalities among recipients in twin-twin transfusion syndrome after fetoscopic laser surgery in 90 consecutive cases. J Med Ultrason (2001). 2020;47(1):117–21.

    Article  Google Scholar 

  14. Mahieu-Caputo D, Dommergues M, Delezoide AL, Lacoste M, Cai Y, Narcy F, et al. Twin-to-twin transfusion syndrome. Role of the fetal renin-angiotensin system. Am J Pathol. 2000;156(2):629–36.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  15. Nizard J, Bonnet D, Fermont L, Ville Y. Acquired right heart outflow tract anomaly without systemic hypertension in recipient twins in twin-twin transfusion syndrome. Ultrasound Obstet Gynecol. 2001;18(6):669–72.

    Article  CAS  PubMed  Google Scholar 

  16. Espinoza J, Furtun BY, Kailin JA, Altman CA, Seaman RD, Belfort MA, et al. Umbilical artery doppler patterns and right ventricular outflow abnormalities in twin-twin transfusion syndrome. J Ultrasound Med. 2021;40(1):71–8.

    Article  PubMed  Google Scholar 

  17. Michelfelder E, Tan X, Cnota J, Divanovic A, Statile C, Lim FY, et al. Prevalence, spectrum, and outcome of right ventricular outflow tract abnormalities in twin-twin transfusion syndrome: a large, single-center experience. Congenit Heart Dis. 2015;10(3):209–18.

    Article  PubMed  Google Scholar 

  18. Roberts D, Neilson JP, Kilby MD, Gates S. Interventions for the treatment of twin-twin transfusion syndrome. Cochrane Database Syst Rev. 2014;30(1):CD002037.

  19. Emery SP, Hasley SK, Catov JM, Miller RS, Moon-Grady AJ, Baschat AA, et al. North American Fetal Therapy Network: intervention vs expectant management for stage I twin-twin transfusion syndrome. Am J Obstet Gynecol. 2016;215(3):346 e341–7.

    Article  Google Scholar 

  20. Khalil A, Cooper E, Townsend R, Thilaganathan B. Evolution of Stage 1 Twin-to-Twin Transfusion Syndrome (TTTS): systematic review and meta-analysis. Twin Res Hum Genet. 2016;19(3):207–16.

    Article  PubMed  Google Scholar 

  21. Rychik J, Tian Z, Bebbington M, Moldenhauer J, Khalek N, Johnson M. Evaluation of the cardiovascular system in twin-twin transfusion syndrome: it's not about ‘scores’ but about ‘goals’. Ultrasound Obstet Gynecol. 2010;36(5):647–8.

    Article  CAS  PubMed  Google Scholar 

  22. Barrea C, Hornberger LK, Alkazaleh F, McCrindle BW, Roberts A, Berezovska O, et al. Impact of selective laser ablation of placental anastomoses on the cardiovascular pathology of the recipient twin in severe twin-twin transfusion syndrome. Am J Obstet Gynecol. 2006;195(5):1388–95.

    Article  PubMed  Google Scholar 

  23. Chang YL, Chao AS, Chang SD, Li WF, Cheng PJ. Predisposing factors and neonatal outcomes for twin-twin transfusion syndrome cases developing transient donor hydrops after fetoscopic laser coagulation: a case control study. BMC Pregnancy Childbirth. 2019;19(1):87.

    Article  PubMed  PubMed Central  Google Scholar 

  24. Stagnati V, Chalouhi GE, Essaoui M, Giuseppi A, Stirnemann JJ, Le Bidois J, et al. Pulmonary stenosis in complicated monochorionic twin pregnancies: prevalence, management and outcome. Prenat Diagn. 2015;35(11):1085–92.

    Article  CAS  PubMed  Google Scholar 

  25. Hoffman JI. Incidence of congenital heart disease: I. Postnatal incidence. Pediatr Cardiol. 1995;16(3):103–13.

    Article  CAS  PubMed  Google Scholar 

  26. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39(12):1890–900.

    Article  PubMed  Google Scholar 

  27. Moon-Grady AJ, Rand L, Lemley B, Gosnell K, Hornberger LK, Lee H. Effect of selective fetoscopic laser photocoagulation therapy for twin-twin transfusion syndrome on pulmonary valve pathology in recipient twins. Ultrasound Obstet Gynecol. 2011;37(1):27–33.

    Article  CAS  PubMed  Google Scholar 

  28. Hecher K, Gardiner HM, Diemert A, Bartmann P. Long-term outcomes for monochorionic twins after laser therapy in twin-to-twin transfusion syndrome. Lancet Child Adolesc Health. 2018;2(7):525–35.

    Article  PubMed  Google Scholar 

  29. Eschbach SJ, Boons L, Van Zwet E, Middeldorp JM, Klumper F, Lopriore E, et al. Right ventricular outflow tract obstruction in complicated monochorionic twin pregnancy. Ultrasound Obstet Gynecol. 2017;49(6):737–43.

    Article  CAS  PubMed  Google Scholar 

  30. Chmait RH, Kontopoulos EV, Korst LM, Llanes A, Petisco I, Quintero RA. Stage-based outcomes of 682 consecutive cases of twin-twin transfusion syndrome treated with laser surgery: the USFetus experience. Am J Obstet Gynecol. 2011;204(5):393 e391–6.

    Article  Google Scholar 

  31. Chang YL, Chao AS, Chang SD, Hsieh PC, Su SY, Chen KJ, et al. Outcome of twin-twin transfusion syndrome treated by laser therapy in Taiwan’s single center: Role of Quintero staging system. Taiwan J Obstet Gynecol. 2016;55(5):700–4.

    Article  PubMed  Google Scholar 

  32. Chang YL, Chao AS, Chang SD, Lien R, Hsieh PC, Wang CN. The neurological outcomes of surviving twins in severe twin-twin transfusion syndrome treated by fetoscopic laser photocoagulation at a newly established center. Prenat Diagn. 2012;32(9):893–6.

    Article  PubMed  Google Scholar 

  33. Hessami K, Nassr AA, Sananes N, Castillo J, Castillo HA, Sanz Cortes M, et al. Prenatal risk factors of neurodevelopmental impairment after fetoscopic laser photocoagulation for twin-twin transfusion syndrome: a systematic review and meta-analysis. Ultrasound Obstet Gynecol. 2021;58(5):658–68.

  34. Daubeney PE, Sharland GK, Cook AC, Keeton BR, Anderson RH, Webber SA. Pulmonary atresia with intact ventricular septum: impact of fetal echocardiography on incidence at birth and postnatal outcome. UK and Eire Collaborative Study of Pulmonary Atresia with Intact Ventricular Septum. Circulation. 1998;98(6):562–6.

    Article  CAS  PubMed  Google Scholar 

Download references

Acknowledgements

Not Applicable.

Funding

No funding was obtained for this study.

Author information

Authors and Affiliations

Authors

Contributions

YLC, ASC, and WFL performed the fetoscopic-guided laser surgery for TTTS. YLC, ASC, and SDC designed the study. YLC, WFL, PJC, and CHC collected the data. SDC, WFL, PJC, and CHC interpreted the results. All authors contributed to the drafting and revision of the manuscript, approved this final version for publication, and are willing to accept responsibility for the accuracy and integrity of its content.

Corresponding author

Correspondence to Yao-Lung Chang.

Ethics declarations

Ethics approval and consent to participate

The study was approved by the Institutional Review Board of Chang Gung Memorial Hospital (IRB #202101274B0). Due to the retrospective nature of this study, written informed consent from the patients was not required. All procedures were conducted in accordance with the ethical standards of the Institutional Review Board of the Chang Gung Medical Foundation and the Declaration of Helsinki.

Consent for publication

Not applicable.

Competing interests

The authors declare that they have no competing interests.

Additional information

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Open Access This article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Chang, YL., Chao, AS., Chang, SD. et al. Incidence, prognosis, and perinatal outcomes of and risk factors for severe twin–twin transfusion syndrome with right ventricular outflow tract obstruction in the recipient twin after fetoscopic laser photocoagulation. BMC Pregnancy Childbirth 22, 326 (2022). https://doi.org/10.1186/s12884-022-04668-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI: https://doi.org/10.1186/s12884-022-04668-1

Keywords