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Pregnancy and early post-natal outcomes of fetuses with functionally univentricular heart in a low-and-middle-income country

Published online by Cambridge University Press:  22 September 2020

Balu Vaidyanathan*
Affiliation:
The Fetal Cardiology Division, Department of Paediatric Cardiology, Amrita Institute of Medical Sciences, AIMS Ponekkara PO, Kochi, Kerala, India682 041
Aparna Vijayaraghavan
Affiliation:
The Fetal Cardiology Division, Department of Paediatric Cardiology, Amrita Institute of Medical Sciences, AIMS Ponekkara PO, Kochi, Kerala, India682 041
Stephy Thomas
Affiliation:
The Fetal Cardiology Division, Department of Paediatric Cardiology, Amrita Institute of Medical Sciences, AIMS Ponekkara PO, Kochi, Kerala, India682 041
Abish Sudhakar
Affiliation:
The Fetal Cardiology Division, Department of Paediatric Cardiology, Amrita Institute of Medical Sciences, AIMS Ponekkara PO, Kochi, Kerala, India682 041
*
Author for correspondence: Dr Balu Vaidyanathan, Clinical Professor, Paediatric Cardiology Head, Fetal Cardiology division, Amrita Institute of Medical Sciences, Kochi, Kerala, India, 682 041. Tel: +91 484 285 3570; Fax: +91 484 280 2020; Mobile: +91 94958 20684. E-mail: [email protected]

Abstract

Background:

Care of children with functionally univentricular hearts is resource-intensive.

Objectives:

To analyse pregnancy and early post-natal outcomes of fetuses with functionally univentricular hearts in the setting of a low–middle-income country.

Methods:

A retrospective study was conducted during the period of January 2008–October 2019. Study variables analysed included gestational age at diagnosis, maternal and fetal comorbidities and cardiac diagnosis including morphologic type of single ventricle. Outcomes analysed included pregnancy outcomes, type of post-natal care and survival status on the last follow-up.

Results:

A total of 504 fetuses were included. Mean maternal age was 27.5 ± 4.8 years and mean gestational age at diagnosis was 25.6 ± 5.7 weeks. Pregnancy outcomes included non-continued pregnancies (54%), live births (42.7%) and loss to follow-up (3.3%). Gestational age at diagnosis was the only factor that impacted pregnancy outcomes (non-continued pregnancies 22.5 ± 3.5 vs. live births 29.7 ± 5.7 weeks; p < 0.001). Of the 215 live births, intention-to-treat was reported in 119 (55.3%) cases; of these 103 (86.6%) underwent cardiac procedures. Seventy-nine patients (36.7%) opted for comfort care. On follow-up (median 10 (1–120) months), 106 patients (21%) were alive. Parental choice of intention-to-.treat or comfort care was the only factor that impacted survival on follow-up.

Conclusions:

Prenatal diagnosis of functionally univentricular hearts was associated with overall low survival status on follow-up due to parental decisions on not to continue pregnancy or non-intention-to-treat after birth. Early detection of these complex defects by improved prenatal screening can enhance parental options and reduce resource impact in low-and-middle-income countries.

Type
Original Article
Copyright
© The Author(s), 2020. Published by Cambridge University Press

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References

van der Linde, D, Konings, EE, Slager, MA, et al. Birth prevalence of congenital heart disease worldwide: a systematic review and meta-analysis. J Am Coll Cardiol 2011; 58: 22412247.CrossRefGoogle ScholarPubMed
Frescura, C, Thiene, G. The new concept of univentricular heart. Front Pediatr 2014; 2: 6265. doi: 10.3389/fped.2014.00062.CrossRefGoogle ScholarPubMed
Steinberger, EK, Ferencz, C, Loffredo, CA. Infants with single ventricle: a population-based epidemiological study. Teratology 2002; 65: 106115.CrossRefGoogle ScholarPubMed
O’Leary, PW. Prevalence, clinical presentation and natural history of patients with single ventricle. Prog Pediatr Cardiol 2002; 16: 3138.CrossRefGoogle Scholar
d’Udekum, Y, Iyengar, AJ, Galati, RG, Forsdick, V, Weintraub, RG, Wheaton, GR. Redefining expectations of long-term survival after the Fontan procedure. Twenty-five years of follow-up from the entire population of Australia and New Zealand. Circulation 2014; 130: (Suppl 1) S32S38.CrossRefGoogle Scholar
Raissadati, A, Nieminen, H, Jokinen, E, Sairanen, H. Progress in late results among pediatric cardiac surgery patients. a population based 6-decade study with 98% follow-up. Circulation 2015; 131: 347353.CrossRefGoogle ScholarPubMed
Schidlow, DN, Gauvreau, K, Cherian, KM, et al. Single-ventricle palliation in Low-and Middle-income countries. J Am Coll Cardiol 2019; 74: 928931.CrossRefGoogle ScholarPubMed
Zentner, D, Celermajer, DS, Gentles, T, et al. Management of people with a Fontan circulation: a cardiac society of Australia and New Zealand Position statement. Heart, Lung and Circ 2020; 29: 539.CrossRefGoogle ScholarPubMed
Wernovsky, G. Current insights regarding neurological and developmental abnormalities in children and young adults with complex congenital cardiac disease. Cardiol Young 2006; 16: (Suppl 1): 92104.CrossRefGoogle Scholar
Kumar, RK, Shrivastava, S. Pediatric heart care in India. Heart 2008; 94: 984990.10.1136/hrt.2007.139360CrossRefGoogle ScholarPubMed
Raj, M, Paul, M, Sudhakar, A, et al. Micro-economic impact of congenital heart surgery: results of a prospective study from a limited-resource setting. PLoS One 2015; 10: e0131348.CrossRefGoogle ScholarPubMed
Carvalho, JS, Allan, LD, Chaoui, R, et al. ISUOG practice guidelines(updated): sonographic screening examination of the fetal heart. Ultrasound Obstet Gynecol 2013; 41: 348359.CrossRefGoogle Scholar
Rychik, J, Ayres, N, Cuneo, B, et al. American Society of Echocardiography guidelines and standards for performance of the fetal echocardiogram. J Am Soc Echocardiogr 2004; 17: 803810.CrossRefGoogle ScholarPubMed
Donofrio, MT, Moon-Grady, AJ, Hornberger, LK, et al. American Heart Association Adults With Congenital Heart Disease Joint Committee of the Council on Cardiovascular Disease in the Young and Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and Council on Cardiovascular and Stroke Nursing. Diagnosis and treatment of fetal cardiac disease: a scientific statement from the American Heart Association. Circulation 2014; 129: 21832242.CrossRefGoogle Scholar
Copel, JA, Pilu, G, Green, J, Hobbins, JC, Kleinman, CS. Fetal echocardiographic screening for congenital heart disease: the importance of the four-chamber view. Am J Obstet Gynecol 1987; 157: 648655.CrossRefGoogle ScholarPubMed
Jicinska, H, Vlasin, P, Jicinsky, M, et al. Does First-Trimester Screening Modify the Natural History of Congenital Heart Disease? Analysis of Outcome of Regional Cardiac Screening at 2 Different Time Periods. Circulation 2017; 135: 10451055.CrossRefGoogle ScholarPubMed
Sonek, JD, Kagan, KO, Nicolaides, KH. Inverted Pyramid of Care. Clin Lab Med 2016; 36: 305317.CrossRefGoogle ScholarPubMed
Bull, C. Current and potential impact of fetal diagnosis on prevalence and spectrum of serious congenital heart disease at term in the UK. British Paediatric Cardiac Association. Lancet 1999; 354: 12421247.CrossRefGoogle ScholarPubMed
Dolk, H, Loane, M, Garne, E. European Surveillance of Congenital Anomalies (EUROCAT) Working Group. Congenital heart defects in Europe: prevalence and perinatal mortality, 2000 to 2005. Circulation 2011; 123: 841849.CrossRefGoogle ScholarPubMed
Levey, A, Glickstein, JS, Kleinman, CS, et al. The impact of prenatal diagnosis of complex congenital heart disease on neonatal outcomes. Pediatr Cardiol 2010; 31: 587597.CrossRefGoogle ScholarPubMed
Beroukhim, RS, Gauvreau, K, Benavidez, OJ, Baird, CW, LaFranchi, T, Tworetzky, W. Perinatal outcome after prenatal diagnosis of single-ventricle cardiac defects. Ultrasound Obstet Gynecol 2015; 45: 657663.CrossRefGoogle ScholarPubMed
Liu, MY, Zielonka, B, Snarr, BS, Zhang, X, Gaynor, JW, Rychik, J. Longitudinal assessment of outcome from prenatal diagnosis through Fontan Operation for over 500 fetuses with single-ventricle type congenital heart disease: The Philadelphia Fetus-to-Fontan Cohort study. J Am Heart Assoc 2018; 7: e009145. DOI: 10.1161/JAHA.118.009145.CrossRefGoogle ScholarPubMed
Khurana, A, Makhija, B, Deka, D, et al Society of Fetal Medicine Practice Guidelines for the second trimester anomalies scan. J Fetal Med 2014; 1: 1115.CrossRefGoogle Scholar
Manual for first trimester medical termination of pregnancy. Issued by: Technical Operations division, Ministry of Health and Family welfare, Government of India, Nirman Bhavan, New Delhi 110011. http://tcw.nic.in/Acts/MTP-Act-1971.pdf Google Scholar
Bill for amendment of the Medical termination of pregnancy Act, 1971. The Gazette of India. CG-DL-E-12032020. www.egazette.nic.in. Accessed 4th July 2020.Google Scholar
Schneider, C, McCrindle, BW, Carvalho, JS, Hornberger, LK, McCarthy, KP, Daubeney, PEF. Development of Z-scores for fetal cardiac dimensions from echocardiography. Ultrasound Obstet Gynecol 2005; 26: 599605.CrossRefGoogle ScholarPubMed
Jenkins, KJ, Correa, A, Feinstein, JA, et al. Non-inherited risk factors and congenital cardiovascular defects: Current knowledge. A scientific statement from the American Heart association council on Cardiovascular Disease in the Young. Circulation 2007; 115: 29953014.CrossRefGoogle Scholar
Nicolaides, KH, Heath, V, Cicero, S. Increased fetal nuchal translucency at 11–14 weeks. Prenat Diagn 2002; 22: 308315.CrossRefGoogle ScholarPubMed
Sharma, R. Revised Kuppuswamy’s socioeconomic status scale: explained and updated. Indian Pediatri 2017; 54: 867870.CrossRefGoogle ScholarPubMed
Tararbit, K, Bui, TT, Lelong, N, Thieulin, AC, Goffinet, F, Khoshnood, B. Clinical and socioeconomic predictors of pregnancy termination for fetuses with congenital heart defects: a population-based evaluation. Prenat Diagn 2013; 33: 179-186.CrossRefGoogle ScholarPubMed
Economic review 2017. State Planning board, Thiruvananthapuram, Kerala, India. http://spb.kerala.gov.in?ER2017/web_e/ch12.php?id=1&ch=12.Google Scholar
National health mission - Hridyam for little hearts. Available at: http://hridyam.in/chd.php. Accessed February 7, 2020.Google Scholar
Deal, BJ, Jacobs, ML. Management of the failing Fontan circulation. Heart 2012; 98: 10981104.CrossRefGoogle ScholarPubMed
Rychik, J. The relentless effects of the Fontan Paradox. Semin Thorac Cardiovasc Surg Pediatr Card Surg Ann 2016; 19: 3743.CrossRefGoogle ScholarPubMed
Raj, M, Sudhakar, A, Roy, R, Champaneri, B, Joy, TM, Kumar, RK. Health-related quality of life in Indian children: a community-based cross-sectional survey. Indian J Med Res 2017; 145: 521529.Google ScholarPubMed
Musa, NL, Hjortdal, V, Zheleva, B, et al The global burden of congenital heart disease. Cardiology in the Young 2017; 27: S3S8.CrossRefGoogle Scholar
Vijayaraghavan, A, Sudhakar, A, Sundaram, KR, Kumar, RK, Vaidyanathan, B. Prenatal diagnosis and planned peri-partum care as a strategy to improve preoperative status in neonates with critical CHDs in low-resource settings: a prospective study. Cardiol Young 2019; 12: 14811488.CrossRefGoogle Scholar
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