Association of Coarctation of Aorta with Turner Syndrome: A Case Report

Musawer Khan¹Sana Imtiaz²Muhammad Shoaib¹Malaika Tariq³Sadia Zahra³Awais Sardar¹Asif Ullah Khan¹Kamil Ahmad Kamil^4*

• ¹Combined Military Hospital, Quetta, Pakistan
• ²SMBZAN Institute of Cardiology, Quetta, Pakistan
• ³Quetta Institute Of Medical Sciences, Quetta, Pakistan
• ⁴Ministry of Public Health (Afghanistan), Kabul, Afghanistan

Monosomy 45,X is commonly associated with congenital heart defects such as coarctation of the aorta (CoA). In this case, the patient developed respiratory distress due to hemodynamic instability from a large bidirectional patent ductus arteriosus (PDA) shunt and systemic hypoperfusion secondary to CoA, which complicated diagnosis and management.We report a 34-week premature female neonate, weighing 1.94 kg, delivered via lower segment cesarean section (LSCS) due to oligohydramnios and intrauterine growth restriction. She exhibited characteristic features of Turner syndrome, including a webbed neck, low-set ears, widely spaced nipples, and lymphedema of the hands and feet. Karyotyping confirmed 45, X.Echocardiography revealed a bicuspid aortic valve, juxtaductal coarctation of the aorta, a moderate-sized PDA with a bidirectional shunt, and suspected pulmonary hypertension. A contrast-enhanced CT aortogram confirmed the coarctation. The patient was managed with mechanical ventilation, CPAP, surfactant therapy, and phototherapy. Rescue transcatheter balloon angioplasty was performed for the coarctation, followed by PDA ligation and surgical coarctation repair at a tertiary center, resulting in marked clinical improvement.