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Surgical Management and Outcome of Patients with Chronic Thromboembolic Pulmonary Hypertension (CTEPH): Results from a European Prospective Registry
Eckhard Mayer1, Jaroslav Lindner2, Andrea M. D'Armini3, David Jenkins4, Walter Klepetko5, Philippe G. Dartevelle6;
1Department of Thoracic Surgery, Kerckhoff Lung Center, Bad Nauheim, Germany; 2Department of Cardiovascular Surgery, Charles University, Prague, Czech Republic; 3Division of Cardiac Surgery, San Matteo Hospital, University of Pavia,, Pavia, Italy; 4Department of Cardiothoracic Surgery, Papworth Hospital, Cambridge, United Kingdom; 5Department of Cardiothoracic Surgery, Medical University of Vienna, Vienna, Austria;
6Department of Thoracic and Vascular Surgery, Marie-Lannelongue Hospital, Le Plessis Robinson, Paris-Sud University, Le Plessis Robinson, France

Objective: Pulmonary endarterectomy (PEA) is the treatment of choice for CTEPH patients who are considered operable by an experienced surgeon and interdisciplinary team. Normal or near-normal cardiopulmonary function and exercise capacity can be restored by surgery. The current real-world management and early postoperative outcome of patients undergoing PEA are described from the European CTEPH registry.
Methods: The prospective registry was designed to include newly diagnosed (≤ 6 months) consecutive patients with CTEPH, from February 2007 until January 2009. Diagnosis was confirmed by right heart catheterization, ventilation-perfusion lung scintigraphy, computerized tomography and/or pulmonary angiography.
Results: 679 patients with CTEPH were registered from 26 European and 1 Canadian centers. Based on surgeon’s assessment, 428 patients (63.5%) were considered operable and 246 (36.5%) non-operable (5 patients missing data). Eventually, 381 patients (56.1%) underwent surgery, 38 refused, 7 died prior to surgery; the median [range] time from CTEPH diagnosis to surgery was 78 days [0-588 days].
Prior to PEA, 99 (26.0%) of the patients were on PH-specific mono therapy (phosphodiesterase type V inhibitor, endothelin receptor antagonist or prostacyclin analogue), and 6 (1.6%) were on dual therapy. PEA was considered “complete” by the operating surgeon in 347 out of 371 evaluable patients (93.5%). The median [range] duration of circulatory arrest was 36 minutes [0-108 min] and median duration of postoperative mechanical ventilation was 1.4 days [0-45 days].
Operative mortality was 4.5 %. Perioperative complications occurred in 182 patients (48.7 %): persistent PH ( mPAP > 25 mmHg or echocardiographic systolic PAP > 40 mmHg) at the end of intensive care (n=63, 16.8%), pulmonary reperfusion edema (n=37, 9.9%), neurological (n=41 (2 irreversible), 11.0%), bleeding (n=38, 10.2%) problems or pericardial effusion (n=31, 8.3%).
Surgical results are summarized in the table for all operated patients, patients with persistent PH and patients who died perioperatively.
Conclusion: The management of CTEPH patients in the participating specialized centers suggests high quality of care as indicated by low operative mortality and good early results. Future follow-up data will support critical decision-making regarding operability and treatment options for these patients.


operated (n = 381) persistent PH (n = 63) dead (n = 17)
Median time from diagnosis to PEA (days) 78 79 108
Vena cava filter or clip (% patients) 39.0 27.9 58.3
Complete PEA (% patients) 93.5 84.1 76.9
Median circulatory arrest time (min) 36 40 40
Median mechanical ventilation time (days) 1.4 2.0 5.0
Median pulmonary vascular resistance at end of intensive care (dyn.s.cm-5) 247 384 398


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