Our Summary

Tetrallogy of Fallot (TOF) is a common type of heart disease that babies are born with. Despite improvements in surgeries and treatments, many patients still die from this condition. One of the main reasons for this is that a part of the heart called the pulmonary valve (PV) often doesn’t work properly after surgery to fix the section of the heart that pumps blood to the lungs. This can lead to serious heart problems and sometimes death.

Over the years, doctors have improved their strategies for replacing the pulmonary valve (PVR). The key to successful PVR is deciding on the right time to do the operation. However, the signs of heart failure are not always clear, so they can’t be relied on to determine the best time for surgery. Instead, doctors use medical imaging to check the condition of the PV and see how it’s working.

Different types of imaging have different uses. For example, a method called transthoracic echocardiography is often used initially and for ongoing checks of patients with TOF, but it has some limitations, especially with adults. More advanced methods, like cardiac computed tomography and magnetic resonance imaging, are now frequently used before and after surgery. These methods can give very detailed information about the structure of the heart and how it’s working.

Doctors are now using evidence from these images to help decide when to do PVR. This article reviews the changes in the heart after TOF surgery, the main things doctors look for in the images, when PVR should be done, and how imaging helps determine the best time for PVR.

FAQs

  1. What is Tetrallogy of Fallot (TOF) and how is it related to pulmonary valve replacement (PVR)?
  2. How does imaging play a role in the assessment of pulmonary valve integrity and dysfunction in TOF patients?
  3. What are the main imaging anatomic and physiologic features in the assessment of patients with TOF?

Doctor’s Tip

A doctor might advise a patient undergoing pulmonary valve replacement to closely follow their post-operative care plan, including attending all follow-up appointments and adhering to any prescribed medications or rehabilitation exercises. It is also important for the patient to communicate any changes in their symptoms or overall health to their healthcare provider. Additionally, maintaining a healthy lifestyle, including regular exercise and a balanced diet, can help support the success of the surgery and overall heart health.

Suitable For

Patients who are typically recommended for pulmonary valve replacement (PVR) include those with Tetralogy of Fallot (TOF) who have residual dysfunction of the pulmonary valve after correction of right ventricular outflow tract obstruction. This dysfunction can lead to irreversible right ventricular dysfunction, arrhythmias, heart failure, and even death. The timing of PVR is crucial in these patients, and imaging plays a key role in assessing PV integrity and dysfunction. Transthoracic echocardiography, cardiac computed tomography, and magnetic resonance imaging are commonly used to evaluate patients with TOF and determine the best timing for PVR. Imaging evidence of disease progression is now part of major guidelines in defining the appropriate timing for reintervention. This article reviews the pathophysiology after TOF repair, identifies imaging features, describes indications for PVR, and highlights the role of imaging in assessing patients with TOF for PVR.

Timeline

Before pulmonary valve replacement (PVR), a patient with Tetrallogy of Fallot (TOF) may experience symptoms such as cyanosis, dyspnea, fatigue, and exercise intolerance due to residual dysfunction of the pulmonary valve. They may undergo various imaging tests such as transthoracic echocardiography, cardiac computed tomography, and magnetic resonance imaging to assess the severity of the PV dysfunction and plan for the surgery.

After PVR, the patient may experience improved exercise tolerance, reduced risk of arrhythmias, and improved overall quality of life. They will require regular follow-up appointments with their healthcare provider to monitor their heart function and assess the success of the surgery. Imaging tests may be repeated to ensure the PV is functioning properly and to detect any potential complications. In some cases, additional interventions may be necessary in the future to maintain the function of the PV and prevent further complications.

What to Ask Your Doctor

  1. What are the potential risks and complications associated with pulmonary valve replacement (PVR)?
  2. How will the PVR procedure be performed and what is the expected recovery time?
  3. How will the function of the new pulmonary valve be monitored in the long term?
  4. What lifestyle changes or restrictions may be necessary after PVR?
  5. Are there any alternative treatment options to PVR that should be considered?
  6. How frequently will follow-up appointments be needed after PVR?
  7. What symptoms should I watch for that may indicate a complication or the need for further intervention?
  8. What is the expected outcome and prognosis after PVR?

Reference

Authors: Flors L, Bueno J, Gish D, White S, Norton PT, Hagspiel KD, Leiva-Salinas C. Journal: J Thorac Imaging. 2020 May;35(3):153-166. doi: 10.1097/RTI.0000000000000478. PMID: 32073541