A Tale Of Two Conversions: Dichotomous Decision In The Spectrum Of Shone'S Complex, Still A Dilemma
Nicholas Oh, Tara Karamlou, Miza Hammoud, Sara Hossieny, Betemariam Sharew, John Costello, Munir Ahmad, Hani Najm.
Cleveland Clinic Foundation, Cleveland, OH, USA.
Purpose: Choosing a management strategy for borderline structures in Shone's complex still poses a challenge. We present two contrasting cases: one with a Fontan circulation that underwent biventricular conversion, and another who underwent a single ventricle palliation (SVP) after failed biventricular attempt by isolated aortic arch repair. Methods: Patient 1: 11-year-old male with mildly hypoplastic left ventricle (LV), critical aortic stenosis, and arcade-like mitral valve (MV) who was palliated to a completion Fontan. He developed severe aortic valve (AV) regurgitation. Evaluation revealed an adequate LV volume (64 cc/m2) and potentially repairable MV. He underwent biventricular conversion including takedown of Fontan and DKS, aorto-ventriculoplasty, pulmonary artery and bicaval reconstruction, AV replacement, MV repair, and resection of endocardial fibroelastosis. Patient 2: 8-week male on initial evaluation demonstrated a small apex forming LV, a small parachute MV (gradient of 1mmHg), and mildly hypoplastic arch with discrete coarctation. Initial coarctation repair relieved the arch obstruction, but MV gradient rose to 11mmHg. Catheterization demonstrated RV hypertension, and elevated left atrial and LV-end-diastolic pressures (gradient 20mmHg). The patient was transferred to our center and underwent a Norwood procedure on day-of-life 23. Results: Both patients had uncomplicated convalescence following conversion. Conclusion: These contrasting cases underscore the importance of appropriate patient selection, biventricular repair timing, and re-evaluation of management strategies. Biventricular conversion should be pursued with optimal cardiac function and hemodynamics, and before developing single ventricle derangements. Likewise, prompt reversal to SVP should be considered when unfavorable biventricular circulation is recognized
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