Therapeutic Effects of Treat and Repair Strategy in Pediatric Patients with Pulmonary Arterial Hypertension and Simple Congenital Heart Defects

Overview

Journal Pulm Circ

Publisher Wiley

Specialty Pulmonary Medicine

Date 2024 May 16

PMID 38751611

Authors

Xiaofeng Wang

Shilin Wang

Zhongyuan Lu

Wenlong Wang

Xu Wang

Affiliations

Soon will be listed here.

Abstract

Surgical indications for patients with pulmonary arterial hypertension (PAH) and congenital heart defects are controversial. The treat and repair strategy has demonstrated efficacy in adult populations, but there have been no studies on pediatric patients. This study included pediatric patients with PAH and simple congenital heart defects who underwent corrective repair between 2012 and 2021. According to the preoperative treatment strategies, the patients were divided into a regular strategy group (Group 1) and a treat-and-repair strategy group (Group 2). Postoperative recovery and follow-up results were compared between the two groups. A total of 33 patients were included in this study. Group 1 consisted of 19 patients, whereas Group 2 consisted of 14 patients. The pulmonary vascular resistance index in Group 2 was higher than that in Group 1 (10.9 ± 4.1 vs. 8.2 ± 1.6 WU, = 0.031). There were no differences in postoperative recovery between the two groups ( > 0.05). During follow-up, five patients were lost (three in Group 1 and two in Group 2). The median follow-up period was 59 months. One patient died in Group 1, and two patients died in Group 2. There was no significant difference in the survival curve ( = 0.39). At the last follow-up, another seven patients had experienced a non-low-risk condition, with a total of three non-low-risk patients in Group 1 and seven in Group 2, including one patient in each group who had a history of ICU admission. According to the ROC curve, a preoperative PVRi <8.2 WU×m can predict postoperative persistent low-risk state, PVRi <5.2 WU×m can avoid postoperative death and/or ICU administration. In pediatric patients with PAH and simple congenital heart defects, the treat and repair strategies may provide surgery opportunities, PVRi should be <8 WU×m, and <5.2 WU×m is the best choice.

Citing Articles

Therapeutic effects of treat and repair strategy in pediatric patients with pulmonary arterial hypertension and simple congenital heart defects.

Wang X, Wang S, Lu Z, Wang W, Wang X Pulm Circ. 2024; 14(2):e12387.

PMID: 38751611 PMC: 11095199. DOI: 10.1002/pul2.12387.

References

Golovenko O, Lazorhyshynets V, Prokopovych L, Truba Y, DiSessa T, Novick W . Early and long-term results of ventricular septal defect repair in children with severe pulmonary hypertension and elevated pulmonary vascular resistance by the double or traditional patch technique. Eur J Cardiothorac Surg. 2022; 62(2). DOI: 10.1093/ejcts/ezac347. View

Tennant P, Pearce M, Bythell M, Rankin J . 20-year survival of children born with congenital anomalies: a population-based study. Lancet. 2010; 375(9715):649-56. DOI: 10.1016/S0140-6736(09)61922-X. View

Ruopp N, Cockrill B . Diagnosis and Treatment of Pulmonary Arterial Hypertension: A Review. JAMA. 2022; 327(14):1379-1391. DOI: 10.1001/jama.2022.4402. View

Bradley E, Ammash N, Martinez S, Chin K, Hebson C, Singh H . "Treat-to-close": Non-repairable ASD-PAH in the adult: Results from the North American ASD-PAH (NAAP) Multicenter Registry. Int J Cardiol. 2019; 291:127-133. DOI: 10.1016/j.ijcard.2019.03.056. View

Pascall E, Tulloh R . Pulmonary hypertension in congenital heart disease. Future Cardiol. 2018; 14(4):343-353. PMC: 6136120. DOI: 10.2217/fca-2017-0065. View

Alonso-Gonzalez R, Lopez-Guarch C, Subirana-Domenech M, Oliver Ruiz J, Otero Gonzalez I, Segovia Cubero J . Pulmonary hypertension and congenital heart disease: An insight from the REHAP National Registry. Int J Cardiol. 2015; 184:717-723. DOI: 10.1016/j.ijcard.2015.02.031. View

Galie N, Humbert M, Vachiery J, Gibbs S, Lang I, Torbicki A . 2015 ESC/ERS Guidelines for the Diagnosis and Treatment of Pulmonary Hypertension. Rev Esp Cardiol (Engl Ed). 2016; 69(2):177. DOI: 10.1016/j.rec.2016.01.002. View

Lammers A, Bauer L, Diller G, Helm P, Abdul-Khaliq H, Bauer U . Pulmonary hypertension after shunt closure in patients with simple congenital heart defects. Int J Cardiol. 2020; 308:28-32. DOI: 10.1016/j.ijcard.2019.12.070. View

Xu Z, Li Q, Zhang C, Zhang H, Gu H . [Risk factors for death and the clinical features of different subtypes of patients with pulmonary arterial hypertension related to congenital heart disease]. Zhonghua Xin Xue Guan Bing Za Zhi. 2020; 48(4):315-322. DOI: 10.3760/cma.j.cn112148-20190628-00364. View

10.

Brida M, Gatzoulis M . Pulmonary arterial hypertension in adult congenital heart disease. Heart. 2018; 104(19):1568-1574. DOI: 10.1136/heartjnl-2017-312106. View

11.

Baumgartner H, De Backer J, Babu-Narayan S, Budts W, Chessa M, Diller G . 2020 ESC Guidelines for the management of adult congenital heart disease. Eur Heart J. 2020; 42(6):563-645. DOI: 10.1093/eurheartj/ehaa554. View

12.

Haworth S, Hislop A . Treatment and survival in children with pulmonary arterial hypertension: the UK Pulmonary Hypertension Service for Children 2001-2006. Heart. 2008; 95(4):312-7. DOI: 10.1136/hrt.2008.150086. View

13.

Takaya Y, Akagi T, Sakamoto I, Kanazawa H, Nakazawa G, Murakami T . Efficacy of treat-and-repair strategy for atrial septal defect with pulmonary arterial hypertension. Heart. 2021; 108(5):382-387. PMC: 8862039. DOI: 10.1136/heartjnl-2021-319096. View

14.

Li Q, Zhang C, Wang R, Keller B, Gu H . Pulmonary hypertensive crisis in children with pulmonary arterial hypertension undergoing cardiac catheterization. Pulm Circ. 2022; 12(2):e12067. PMC: 9063957. DOI: 10.1002/pul2.12067. View

15.

Akagi S, Kasahara S, Sarashina T, Nakamura K, Ito H . Treat-and-repair strategy is a feasible therapeutic choice in adult patients with severe pulmonary arterial hypertension associated with a ventricular septal defect: case series. Eur Heart J Case Rep. 2019; 2(2):yty033. PMC: 6176981. DOI: 10.1093/ehjcr/yty033. View

16.

Hu Z, Xie B, Zhai X, Liu J, Gu J, Wang X . Midterm results of "treat and repair" for adults with non-restrictive ventricular septal defect and severe pulmonary hypertension. J Thorac Dis. 2015; 7(7):1165-73. PMC: 4522505. DOI: 10.3978/j.issn.2072-1439.2015.07.06. View

17.

Fathallah M, Krasuski R . A Multifaceted Approach to Pulmonary Hypertension in Adults With Congenital Heart Disease. Prog Cardiovasc Dis. 2018; 61(3-4):320-327. DOI: 10.1016/j.pcad.2018.07.017. View

18.

Hansmann G, Koestenberger M, Alastalo T, Apitz C, Austin E, Bonnet D . 2019 updated consensus statement on the diagnosis and treatment of pediatric pulmonary hypertension: The European Pediatric Pulmonary Vascular Disease Network (EPPVDN), endorsed by AEPC, ESPR and ISHLT. J Heart Lung Transplant. 2019; 38(9):879-901. DOI: 10.1016/j.healun.2019.06.022. View

19.

Xie F, Quan R, Zhang G, Tian H, Chen Y, Yu Z . Characteristics, treatments and survival of pulmonary arterial hypertension associated with congenital heart disease in China: Insights from a national multicenter prospective registry. J Heart Lung Transplant. 2023; 42(7):974-984. DOI: 10.1016/j.healun.2023.02.1494. View

20.

Wang X, Wang S, Lu Z, Wang W, Wang X . Therapeutic effects of treat and repair strategy in pediatric patients with pulmonary arterial hypertension and simple congenital heart defects. Pulm Circ. 2024; 14(2):e12387. PMC: 11095199. DOI: 10.1002/pul2.12387. View