CASE REPORT
A 27-year-old male patient was
admitted for palpitation and exertional dyspnea. He was diagnosed with
absent pulmonary valve syndrome (APVS) with an intact ventricular septum
(IVS) and tricuspid regurgitation at the age of
18
years. However, no medical intervention was taken during these years due
to the mild symptom.
This time, an electrocardiogram revealed atrial fibrillation,
noncomplete right bundle branch block, right ventricle hypertrophy, and
abnormal T wave. A transthoracic echocardiogram revealed concordant
atrioventricular and ventriculoarterial connections and an intact
ventricular septum. Both ventricles demonstrated a decreased systolic
function (Figure 1a) , and the right heart was significantly
dilated due to severe tricuspid valve regurgitation (Figure
1b) . A rudimentary pulmonary valve (pulmonary valve annular of 4 mm)
with severe regurgitation of its orifice was observed, and the pulmonary
branches were not dilated yet (Figure 1c) . Color and spectral
Doppler demonstrated the to-and-fro blood across the dysplastic
pulmonary valve (Pressure gradient of 75 mmHg and peak velocity of 432
cm/s) (Figure 1d) .
He underwent pulmonary valve implantation and tricuspid valvoplasty in
our hospital. A significant delimitation was seen between the normal
cardiac muscle and proliferative fibrous tissue under intraoperative
view (Figure 2) . The pulmonary valve leaflets were absent and
substituted with a restrictive ring of thickened tissue. Further, the
tricuspid valve annular was dilated. Surprisingly, we discovered an
absent superior vena cava in this patient. Vein intubation during
cardiopulmonary bypass (CPB) was only obtained in inferior vena cava. He
was successfully weaned off CPB and discharged from the intensive care
unit on postoperative day 4 and went home on postoperative day 22. He
was doing well on the 3-month follow-up. Additionally, the transthoracic
echocardiogram reflected a significant right heart reduction as well as
a systolic function improvement in both ventricles.