• Ayesha Butt Yale School of Medicine, New Haven, CT, USA
  • Naela Ashraf Liaquat National Hospital, Karachi, Pakistan
  • Khuzaima Tariq Liaquat National Hospital, Karachi, Pakistan
  • Muneer Amanullah Liaquat National Hospital, Karachi, Pakistan




Williams-beuren syndrome, supra valvular aortic stenosis, infectious aortitis, valvular lesion


Williams syndrome (WS), is a multisystem disorder occurring in 1 in 10,000 live births with supravalvular aortic stenosis (SVAS) being the most common cardiovascular manifestation. We present the case of a 2.5 years old male, known case of WS who presented with cognitive delay, history of right-sided stroke and left hemiplegia. Echocardiography revealed severe SVAS with a gradient of 105 mmHg. Diameter of the Sino tubular junction was 4 mm. Computerized tomography angiogram showed diffuse stenosis of ascending aorta with intraluminal thrombus. At surgery, the ascending aorta was augmented with autologous pericardial patches and end-to-end anastomosis of the proximal and distal aorta completed the reconstruction. The patient was discharged in a stable condition. He presented 6 weeks post-op with a pulsating pseudoaneurysm through the sternal wound. Emergency surgery with removal of fungal vegetation and reconstruction of the ascending aorta was performed. He expired due to fungal sepsis a week later.

Author Biographies

Ayesha Butt, Yale School of Medicine, New Haven, CT, USA

Postdoctoral Associate, Section of Hematology.

Naela Ashraf, Liaquat National Hospital, Karachi, Pakistan

Research Associate, Department of Cardiothoracic Surgery.

Khuzaima Tariq, Liaquat National Hospital, Karachi, Pakistan

Senior Registrar, Department of Cardiothoracic Surgery.

Muneer Amanullah, Liaquat National Hospital, Karachi, Pakistan

Professor and Consultant, Department of Cardiothoracic Surgery.


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