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.