Shone's Syndrome and Transplant: A Road Less Traveled

Area of Science:

• Cardiology
• Congenital Heart Disease
• Advanced Cardiac Therapies

Background:

• Shone's syndrome is a rare congenital heart disease characterized by four lesions causing left ventricular inflow and outflow obstruction.
• This condition leads to lifelong hemodynamic challenges, frequently necessitating advanced treatment modalities.

Observation:

• This case series examines eight patients with Shone's syndrome referred for advanced therapy evaluation.
• Six patients received heart transplants, while two underwent dual organ transplants.
• Two transplant recipients did not survive post-procedure.

Findings:

• Transplant decisions for Shone's syndrome involve complex considerations, including pulmonary hypertension and the need for single versus dual organ transplantation.
• Patients experienced intricate pre- and postoperative courses.

Implications:

• Early referral for advanced therapies is vital for patients with Shone's syndrome.
• Multidisciplinary evaluation and individualized care plans are essential for optimizing outcomes in this complex patient cohort.