Title: Successful Delivery in a High-Risk Primigravida with Severe Bicuspid Aortic Valve Stenosis with Marfanoid Features: Case Report from a Tertiary Care Centre By Dr. Kavita Ghadale

Introduction:

• Marfan Syndrome and Marfanoid feature as a high-risk patient subset.
• Valvular heart disease in these patients increases delivery risks manifold.
• Challenges associated with severe left ventricular outflow obstruction during delivery.
• The significance of aortic dissection, rupture, and peri-partum cardiomyopathy in patients with Marfanoid habitus.
• We present a unique case of a primigravida patient with Marfanoid features, contractual arachnodactyly, long arms and legs, breech presentation, bicuspid aortic valve with severe Aortic Stenosis, and abruptio placenta.

Methods:

• Clinical presentation and examination findings of the patient.
• Obstetric ultrasound and its role in confirming the diagnosis.
• Cardiac assessment using 2D Echocardiography to determine the severity of aortic stenosis.

Results:

• Impact of pregnancy-related hemodynamic changes on peripartum complications.
• Clinical presentation and emergency department admission at 31 weeks gestation.
• Successful management of the patient through Caesarean Section and neonatal outcomes.
• Confirmation of diagnosis through genetic testing.

Discussion:

• Understanding contractual arachnodactyly and its implications on various body parts.
• Cardiac manifestations, such as mitral valve prolapse and aortic root involvement.
• Importance of baseline echocardiogram and trimester-wise monitoring during pregnancy.
• Differential diagnosis considerations, ruling out Velo-Cardio-Facial Syndrome (VCFS).
• Successful management of a patient with congenital heart disease and Marfanoid features during pregnancy.

Conclusion:

• Recognizing and monitoring rare entities like this high-risk case.
• Ensuring comprehensive cardiac assessment and monitoring during pregnancy.
• Highlighting the unique features and differential diagnosis considerations.
• Successful management of a patient with congenital heart disease and Marfanoid features during pregnancy.