A 66-year-old woman complaining hemoptysis was referred to our hospital. Enhanced chest computed tomography (CT) showed a solid mass measuring 6.2 cm in maximal diameter in the left-upper lobe (LUL) (Fig. 1a, b) accompanied by the narrowing of the LUL pulmonary artery and bronchus surrounded by the mass. Bronchoscopy findings showed that the left-upper bronchus was completely obstructed by the polypoid tumor (Fig. 1c). Three-dimensional CT angiography (Fig. 2a) and enhanced CT (Fig. 2b: coronal view, Fig. 2c: axial view) revealed a PAPVC in the right-upper lobe (RUL) returning into the superior vena cava (SVC).
The mass was pathologically confirmed to be non-small cell lung cancer (NSCLC) by a transbronchial lung biopsy. Her disease was diagnosed as cT3N1M0, stage IIIA, NSCLC. Transthoracic echocardiography showed no evidence of other cardiovascular abnormalities, such as atrial septal defect (ASD).
A pre-treatment cardiac catheterization test showed the Qp/Qs to be 2.24. To avoid fatal right-heart failure after pulmonary resection, we decided to perform correction of the PAPVC in the RUL prior to pulmonary resection. After induction chemoradiation therapy (2 cycles of cisplatin plus vinorelbine with concurrent 40-Gy radiotherapy), the LUL mass showed a partial response according to the response evaluation criteria in solid tumor (Fig. 3a: 3.2 cm in largest diameter, − 49% in size). Correction of the PAPVC in the RUL was performed by cardiovascular surgeons under cardiopulmonary bypass (double-decker method) via median sternotomy [2]. Figure 4 shows the surgical schema of the correction of PAPVC in this patient. The total operation time and the pump time was 259 min and 143 min. The patient required 8 units of red blood cell transfusion intraoperatively. The enhanced chest CT (coronal view) after surgical repair of the PAPVC in the RUL (Fig. 3b) showed the RUL pulmonary vein draining into the left atrium. Thereafter, left-upper lobectomy with bronchoplasty and pulmonary arterial angioplasty was performed. Figure 5 shows the time schedule from induction chemoradiation therapy to pulmonary resection in this patient.
The postoperative course was uneventful except for paroxysmal atrial fibrillation. A pathologic examination of the resected specimen showed that almost 50% of solid predominant adenocarcinoma cells were viable (ypT1cN1M0, stage IIB) with a negative bronchial margin. After pulmonary resection, the patient received no adjuvant therapy. The patient remains alive without recurrence of disease or symptoms of heart failure 17 months after pulmonary resection. So far, the patient also continues to take anti-coagulation therapy (warfarin) after the surgical correction of PAPVC.