THE “MUSTARD DILEMMA”: URGENT NEED FOR A NATIONAL STRATEGY TO TRACK COMPLEX CONGENITAL CARDIAC PATIENTS

Abstract

51 years ago, the first Mustard operation was performed at our institution - and was successful; the patient is still alive today. The procedure’s success was such that many thought they were “cured”. They were not; “failing Mustards” with severe RV failure, TR and often pulmonary hypertension present to us with increasing frequency. We reviewed our experience with an emphasis on this “Mustard dilemma”. In 2010-2013, we undertook multiple iterations of cross-sectional follow-up of all 546. Mustard procedures as part of a “double-switch” operation were not included. All points of last known contact were pursued via as many avenues (primary cardiologists, surgeons, institutions, family physicians). Institutional data protection policies were extremely difficult obstacles, as were breaks in follow-up continuity caused by physician retirement or death. The 546 Mustards (1963 - 2007) were performed at mean age 2.1 years for diagnoses including predominantly variants of arterial transposition or DORV. Most had undergone prior palliation via atrial septectomy (249), systemic-pulmonary shunt (19), PA band (44) or cavopulmonary connection (3). DHCA was used in ∼60% (mean 55 minutes). In-hospital mortality was 9%. Following discharge, late survival was excellent, with very slow rising late hazard for death over the subsequent decades (instantaneous risk ∼1%/ year after 40 years). Estimated survival 50 years after surgery is ∼46% overall (but 58% for isolated TGA). There have been 184 known deaths (33%). Of the 372 presumed survivors, the median age today would be 36 years (IQ range 30 - 40). More than 20% of adult Mustards we follow have decompensated heart failure by age 36, and this proportion rises disproportionately thereafter (figure). At last follow-up, prevalence of ≥ moderate TR was 20% and RV dysfunction 34%. Nevertheless, follow-up with a cardiologist is known for only 50% (182/372) of presumed survivors since 2010, and 60% (224/372) since 2000. The remaining 148 (40% of survivors) only had last known points of cardiology contact in the 1980s or 1990s. The bulk of adult Mustard survivors are approaching high-risk periods for onset of heart failure. The insidious onset of RV dysfunction, TR and pulmonary hypertension emphasizes the need for national tracking registry for this and other complex CHD patients, similar to those for cancer patients, to prevent patients re-presenting with excessive risk for any intervention, including transplantation.