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The Management of a 30-Year-Old Female with Neglected VSD Repair in Childhood
Ariza Martinez, Patrick T. Roughneen
University of Texas Medical Branch, Galveston, TX, USA

OBJECTIVE: We present a case of a large peri-membranous VSD left untreated in a 30-year-old female. Paradoxically, the right ventricular (RV) hypertrophy in the infundibulum was so significant that it prevented the development of Eisenmenger syndrome although she was initially thought to be inoperable.
METHODS: We performed a right ventriculotomy to assess the VSD and RV outflow tract (RVOT) obstruction. This provided good exposure of the VSD and RV hypertrophy. Following ventriculotomy, infundibular hypertrophy was noted, and the obstructing muscle bands were resected. The VSD was closed through a trans-ventricular approach using a 1-mm tissue patch. RVOT was repaired using bovine pericardium. We used a right atrial approach to assess the repaired VSD and ensure no presence of residual VSD and wide patency of RVOT. She was then successfully weaned off bypass with no complications. Post-operatively, we used 21% FiO2 to prevent against pulmonary vasodilatation and pulmonary edema after relief of RVOT obstruction. The hospital course was complicated by mild pulmonary edema, which resolved with diuretics.
RESULTS: The patient’s hospital stay was 7 days. She is currently alive and well 42 months after surgery and is NYHA Class I with no activity restriction.
CONCLUSIONS: This case highlights the unusual presentation of a long-standing VSD with infundibular hypertrophy from pressure and volume overload and was protected from development of Eisenmenger syndrome because of limitation of pulmonary blood flow. Although she was cyanotic on presentation from right-to-left shunting resulting from infundibular hypertrophy, this was not a contraindication to corrective surgery.


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