From the Research
Approximately 85-90% of patients with tetralogy of Fallot (TOF) and pulmonary atresia survive to adulthood with modern surgical and medical management. This survival rate has dramatically improved over the past few decades due to advances in surgical techniques, perioperative care, and long-term management strategies. Patients typically undergo staged surgical repairs, often beginning with placement of a systemic-to-pulmonary shunt in infancy, followed by complete repair in early childhood. The complete repair usually involves ventricular septal defect closure and establishing continuity between the right ventricle and pulmonary arteries, often using conduits or homografts. These patients require lifelong cardiology follow-up with regular echocardiograms, cardiac MRIs, and sometimes cardiac catheterizations to monitor for complications such as conduit stenosis or regurgitation, arrhythmias, and right ventricular dysfunction. Many patients will need additional interventions throughout their lifetime, including pulmonary valve replacements and treatment for arrhythmias. Despite these challenges, with appropriate medical care and timely interventions, the majority of patients can expect to reach adulthood and maintain a good quality of life, as supported by a study published in 2019 1.
Some key points to consider in the management of TOF with pulmonary atresia include:
- The importance of early surgical intervention to establish adequate pulmonary blood flow and prevent long-term complications
- The need for lifelong cardiology follow-up to monitor for potential complications and intervene early when necessary
- The potential for re-operations and interventions throughout the patient's lifetime, including pulmonary valve replacements and treatment for arrhythmias
- The impact of risk factors such as older age at repair, non-sustained ventricular tachycardia, and left ventricular ejection fraction <50% on long-term outcomes, as identified in the study by 1.
Overall, with modern surgical and medical management, the majority of patients with TOF and pulmonary atresia can expect to survive to adulthood and maintain a good quality of life, as demonstrated by the study published in 2019 1.