Sequential Subvalvular and Valvular Stenoses in Pregnancy with Congenital Heart Disease: When It Rains, It Pours

F. Löffler (Hannover)1, M. Westhoff-Bleck (Hannover)2, S. Uehlein (Hannover)1, C. von Kaisenberg (Hannover)3, L. Brodowski (Hannover)4, S. Greve (Hannover)5, S. Heiderich (Hannover)5, B. Bohnhorst (Hannover)6, A. Horke (Hannover)7, M. Avsar (Hannover)7, A.-S. Silber-Peest (Hannover)1, J. Bauersachs (Hannover)1
1Medizinische Hochschule Hannover Kardiologie und Angiologie Hannover, Deutschland; 2Medizinische Hochschule Hannover Kardiologie und Angiologie, EMAH-Zentrum Hannover, Deutschland; 3Medizinische Hochschule Hannover Hannover, Deutschland; 4Department of Gynecology and Obstetrics Hannover, Deutschland; 5Department of Anesthesiology Hannover, Deutschland; 6Department of Pediatrics Hannover, Deutschland; 7Medizinische Hochschule Hannover Klinik für Herz-, Thorax-, Transplantations- und Gefäßchirurgie, OE 6217 Hannover, Deutschland

Objectives
Little is known about pregnancy outcomes in women with sequential valvular and subvalvular stenoses due to congenital heart disease. These lesions limit cardiac adaptation to increased circulatory demands. This case series illustrates associated risks and management strategies to optimize maternal and fetal outcomes.

Methods
We reviewed four pregnant women with complex sequential stenotic lesions managed at our tertiary center: two with mitral and dynamic subaortic stenosis, one with mitral prosthetic obstruction causing left ventricular outflow tract obstruction, and one with combined subvalvular and valvular pulmonary stenosis. Clinical presentation, echocardiographic findings, treatment approaches, and maternal-fetal outcomes were analyzed.

Results
Case 1: A 26 year-old woman with a prior Konno procedure and tissue-engineered aortic valve developed progressive subaortic and mitral stenosis with worsening dyspnea and angina. A preterm cesarean delivery at 23+6 weeks was performed, with favorable maternal and neonatal outcomes, followed by planned valve surgery postpartum.
Case 2: A 28 year-old woman with recurrent subaortic and severe mitral stenosis experienced progressive symptoms and hemodynamic deterioration at 29 weeks. Cesarean delivery at 29+0 weeks led to postpartum improvement, with elective Konno and mitral valve replacement planned. The neonatal outcome was favorable.
Case 3: A 37 year-old woman with a bioprosthetic mitral valve showing mild stenosis and causing fixed moderate LVOTO with a maximum pressure gradient of 37 mmHg underwent a cesarean section at 39 + 0 weeks of gestation, resulting in good maternal and neonatal outcomes.
Case 4: A 24 year-old woman with pulmonary valvular and subpulmonary stenoses presented early in pregnancy with NYHA III symptoms and systemic right ventricular pressure. A percutaneous intervention was attempted but proved insufficient in treating the subpulmonary stenosis. Due to persistent symptoms, pregnancy was terminated at 9+6 weeks of gestational age, and subsequent pulmonary valve replacement with right ventricular outflow tract patch reconstruction was planned.

Conclusion
Pregnancy in women with sequential stenotic lesions carries a substantial maternal and fetal risk, particularly in the presence of dynamic subvalvular obstruction. Even mild stenotic lesions may lead to clinical deterioration under the hemodynamic stress of pregnancy. Early recognition, close multidisciplinary monitoring, individualized delivery planning, and preparedness for advanced interventions are essential.