Abstract
Background: There is limited literature written on the course and outcomes for pregnant mothers with Shone complex. Methods: We describe a case series of five pregnancies in four women with Shone complex within a multidisciplinary cardio-obstetrics clinic from 2016–2018. Results: Maternal age ranged from 21–39 years. Three patients had preserved left ventricular function while one had moderately decreased function. Gestational age at presentation ranged from 6–15 weeks. There were three successful pregnancies (mean gestational age = 37 weeks, range 35–39 weeks) with one patient accounting for two unsuccessful pregnancies. All infants were delivered via Cesarean section. One infant required a NICU stay, but all other infants delivered were healthy. Conclusion: Patients with Shone complex can have successful pregnancies although complications can occur for both the mother and the baby. Comprehensive prenatal care, coordinated and consistent management during pregnancy, and tertiary care support can promote positive maternal and fetal outcomes.
Highlights
Pregnancy for patients with complex congenital heart diseases can be fraught with danger and uncertainty, yet for many women it remains a highly desired experience
Shone complex (Fig. 1) is a rare congenital disorder of multiple left-sided, obstructive cardiac abnormalities consisting of a parachute mitral valve, a supravalvular mitral ring, subvalvular and valvular aortic stenosis, and aortic coarctation [1]
The aim of this paper is to expand current knowledge on Shone complex by reporting a case series of patients seen at a multidisciplinary cardio-obstetrics clinic dedicated to managing pregnancy in patients with cardiovascular disease
Summary
Pregnancy for patients with complex congenital heart diseases can be fraught with danger and uncertainty, yet for many women it remains a highly desired experience. Shone complex (Fig. 1) is a rare congenital disorder of multiple left-sided, obstructive cardiac abnormalities consisting of a parachute mitral valve, a supravalvular mitral ring, subvalvular and valvular aortic stenosis, and aortic coarctation [1]. Panel A: Depiction of the complete Shone complex including a supravalvular mitral ring (black arrow), a parachute mitral valve (red arrow), subaortic stenosis (green arrow), and aortic coarctation (blue arrow). Panel C: Echocardiography of aortic lesions in a patient with Shone complex. Of the five case reports we identified describing pregnant patients with Shone complex, four of these described successful pregnancies with no apparent symptom exacerbation (Table 1). The aim of this paper is to expand current knowledge on Shone complex by reporting a case series of patients seen at a multidisciplinary cardio-obstetrics clinic dedicated to managing pregnancy in patients with cardiovascular disease. The case series is accompanied by a brief literature review and our recommendations
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