Valvuloplasty in woman with heterotaxy syndrome
Richmond R, Shaikh A, Allaqaband S, Ohair D. Valvuloplasty In Woman With Heterotaxy Syndrome. Journal of the American College of Cardiology. 2017;69(11):2216. doi:10.1016/s0735-1097(17)35605-x.
Session Title: FIT Clinical Decision‐Making: Prevention, Adult Congenital and Congenital Heart Disease Location: American College of Cardiology 66th Annual Scientific Session Friday, March 17, 2017, 3:45 p.m.-4:30 p.m. Abstract Category: Congenital Heart Disease Presentation Number: 1169-371 Poster Contributions Poster Hall, Hall C
BACKGROUND: Anatomic variations can make structural interventions challenging and require a more creative approach. Persistent left superior vena cava is an uncommon entity; even more uncommon is a left-sided inferior vena cava connecting to the persistent superior vena cava with eventual drainage to the coronary sinus. Heterotaxia is another twist on normal anatomy, and rarer still.
CASE: A 77-year-old woman with chronic atrial fibrillation and New York Heart Association (NYHA) functional class III heart failure symptoms requiring supplemental oxygen underwent transthoracic echocardiography and was noted to have severe rheumatic mitral stenosis (resting mean gradient of 10 mmHg, valve area <1 cm2 ), severe pulmonary hypertension and a severely dilated left atrium. Left heart catheterization showed no significant coronary artery disease. Right heart catheterization via a right femoral approach delineated a left-sided inferior vena cava that drained into a left-sided superior vena cava with drainage into the coronary sinus. Computed tomography showed heterotaxy syndrome: polysplenia, midline liver, right-sided stomach and left-sided inferior vena cava. Given her severe, symptomatic mitral stenosis, balloon valvuloplasty was needed.
DECISION‐MAKING: Traditionally, mitral balloon valvuloplasty is performed via a transseptal approach. Given her uncharacteristic vascular anatomy, this was deemed high risk. Prior accounts of mitral interventions via a transapical approach have been described, and this approach was deemed a safer method in this case. Therefore, under transesophageal and fluoroscopic guidance, she underwent mitral valvuloplasty via a transapical route. Multiple balloon inflations were performed successfully; her postprocedure gradient was only 5 mmHg, her heart failure symptoms improved to NYHA class I, and she was weaned from supplemental oxygen.
CONCLUSIONS: This case illustrates unconventional anatomical variations, rarely encountered as individual entities, let alone in the same patient. Structural interventions, as described in this case, can still be safely undertaken with careful planning and consideration for alternative approaches.