Abbreviations and Acronyms
14-year-old girl was referred to the cardiology clinic for worsening dyspnea and lower-limb edema. She had a history of Ebstein's malformation associated with bicuspid aortic valve, coarctation of aorta, perimembranous ventricular septal defect (VSD), and subvalvular fibromuscular aortic stenosis. She had undergone coarctation of aorta repair, subaortic fibromuscular stenosis resection, and VSD patch closure during the first months of her life.
A Doppler color echocardiogram showed signs of Ebstein's disease (Carpentier type C) (Fig. 1) with severe tricuspid valve incompetence, mild left ventricular dysfunction (42%), bicuspid aortic valve with mild incompetence, and moderate residual VSD. Because of her clinical deterioration (New York Heart Association functional class III), she underwent surgical tricuspid valve replacement using a biological prosthesis (Hancock II 25 mm; Medtronic). Her postoperative course was complicated; volume/pressure overload through the residual VSD was initially considered and the defect percutaneously closed. Nevertheless, she continues to have pronounced symptoms (dyspnea, edema, and mild cyanosis) related to the large tricuspid paravalvular leak (PVL) measuring 12 mm × 10 mm (Fig. 2 and 3).
The PVL was percutaneously approached via the right internal jugular vein, and a 18-mm × 10-mm rectangular PVL device (Occlutech) was implanted with uneventful results with regard to closing the defect (Fig. 4 and 5). After 3 months, the patient's functional class had improved, and a trivial residual shunt was visualized (Fig. 6).
Ebstein's malformation is a rare and complex congenital lesion. Surgical treatment is recommended when progressive cardiomegaly, exercise intolerance, cyanosis, paradoxical embolism, or arrhythmias (atrial or ventricular) are observed. Tricuspid valve replacement is necessary when repair is not feasible or has failed. Frequently, a biological prosthesis is preferred to a mechanical valve because of its lower thrombogenicity, mainly when right ventricular failure is present.1
The success of interventions for tricuspid PVL in congenital heart disease is anecdotal.2,3 As an alternative to surgery, transcatheter occlusion has often been performed for mitral and aortic paravalvular leaks.4 This case illustrates that a percutaneous approach for closure of a tricuspid PVL is feasible and may be a reasonable alternative to surgery in select patients.
Conflict of Interest Disclosures: No relationship exists with industries related to this work.
Funding/Support: No financial support is involved with this case report.