PURPOSE: Tricuspid regurgitation (TR) in pulmonary arterial hypertension (PAH) is a well recognized corollary of right ventricle (RV) failure. Right ventricular systolic pressure (RVSP) greater than 55mm Hg usually results in functional TR. Isolated tricuspid valve prolapse (TVP) is a rarely described clinical entity. We describe three PAH subjects who developed acute decompensated right heart failure due to TVP with a poor clinical outcome in two.
METHODS: Echocardiograms are part of routine assessment of PAH subjects at the Baylor PH Center. In 5 PAH subjects with acute worsening of RV failure, echocardiogram demonstrated free TR. Of these 5 patients, 2 had ruptured chordae tendinae and 1 had a completely flail leaflet with severely dilated tricuspid annulus.
RESULTS: Two female (F) subjects had idiopathic PAH (ages 36) and one male (M) subject had PAH associated with HIV (age 51) with normal CD4 counts. PAH therapy was optimized with initiation (two subjects) or increases in intravenous prostanoid (one subject) on a background of endothelin receptor antagonist (2) and phosphodiesterase-5 inhibitor therapy (2). 2 subjects (1M & 1F) developed progressively worse ascites, and syncope in spite of optimum therapy. Male PAH subject, who was initially started on intravenous prostanoid therapy, underwent TV replacement and survived for 7 months before succumbing to progressive right heart failure. The second patient (F) who developed TVP despite being on intravenous prostanoid therapy underwent heart-lung transplantation in a debilitated condition due to worsening PAH and expired post-transplant. The third PAH patient (F) is alive and clinically improved (6 minute walk distance = 488 meters) since initiation of intravenous prostanoid for the last 21 months in spite of continued signs of RV failure.
CONCLUSION: Anatomic failure of the tricuspid valve, though rare should be considered in evaluation of sudden RV decompensation in patients with PAH. This can sometimes be stabilize with aggressive PAH therapy but the outcome in our experience is poor.
CLINICAL IMPLICATIONS: TVP should be considered in PAH subjects with sudden RV decompensation.
DISCLOSURE: Sanjeev Raman, None.