From the Guidelines
Treatment for Shone's complex typically involves a combination of medical management and surgical interventions tailored to the specific cardiac defects present, with a focus on correcting multiple left-sided obstructive lesions through staged surgeries. The management of patients with complex severe valvular heart disease, such as Shone's complex, is best achieved by a Heart Valve Team composed primarily of a cardiologist and surgeon, as recommended by the 2014 AHA/ACC Valvular Heart Disease Guideline 1. Initially, medications such as diuretics, ACE inhibitors, and digoxin may be used to manage heart failure symptoms and control blood pressure. However, definitive treatment almost always requires surgical correction of the multiple left-sided obstructive lesions, which may include:
- Mitral valve repair or replacement
- Resection of subaortic stenosis
- Correction of coarctation of the aorta
- Enlargement of a hypoplastic aortic arch The optimal care of the patient with complex heart disease is best performed in centers that can provide all available options for diagnosis and management, including the expertise for complex aortic or mitral valve repair, aortic surgery, and transcatheter therapies, as outlined in the 2014 AHA/ACC Valvular Heart Disease Guideline 1. Timing of surgery depends on symptom severity, with some patients requiring intervention in infancy while others may be managed until later childhood. Following surgical correction, patients need lifelong cardiology follow-up with regular echocardiograms to monitor for recurrent obstruction, valve function, and development of pulmonary hypertension 1. Many patients will require multiple surgeries throughout their lifetime as they grow or if complications develop. Early diagnosis and intervention are crucial for improving long-term outcomes in this complex congenital heart condition.
From the Research
Treatment for Shone's Complex
The treatment for Shone's complex typically involves surgical repair of the obstructive lesions. According to 2, a single-stage surgical repair can be successful in patients with incomplete forms of Shone's complex.
- Surgical procedures may include:
- Coarctation repair
- Mitral valve repair or replacement
- Ventricular septal defect closure
- Subaortic resection
- Complex left ventricular outflow tract reconstruction or bypass 3
- The age at first operation can range from 7 days to 7 years, with a median age of 3 months 3
- In some cases, heart transplantation may be required, as reported in 4, where 2 patients (7.1%) underwent heart transplantation at ages 22 and 28 years
Interventional Catheterization and Surgery
Interventional catheterization and surgery are common interventions for Shone's complex. According to 5, 16% of patients underwent interventional catheterization, while 76% underwent surgery.
- Echocardiography plays a crucial role in the diagnosis and planning of appropriate management for Shone's complex 6
- Cardiac multi-slice CT (MSCT) and transesophageal echocardiography may also be used to guide diagnosis and intervention 5
Outcomes and Morbidity
The outcomes and morbidity associated with Shone's complex can be significant. According to 4, 48% of patients had cardiovascular hospitalizations during adulthood, predominantly for arrhythmias or heart failure.