Abstract
Chronic thromboembolic pulmonary hypertension (CTEPH) is a rare disease which results from obstruction of the major pulmonary arteries by incompletely resolved or organized pulmonary emboli which have become incorporated into the pulmonary artery wall, eventually causing an increase in pulmonary vascular resistances. Pulmonary endarterectomy (PEA) is the treatment of choice. Careful pre- and post-operative management is essential for a successful outcome following PEA. In 1994, we started in Pavia a program in which members of a multidisciplinary team work in close interaction with the aim of increase experience in the challenging problems these patients present in the evaluative, surgical, and post-operative phases of their care. So far, 134 PEAs have been performed. Preoperatively, New York Heart Association (NYHA) class distribution was respectively 3-II, 56-III, and 75-IV; mean pulmonary artery pressure and pulmonary vascular resistances were 47 ± 13 mmHg and 1149 ± 535 dynes/sec/cm−5 respectively. The overall operative mortality has been 9.7% (in 2005 mortality rate was 4.5%). At present, 92% of the PEA patients are actively participating in the follow-up study. Follow-up visits are at 3 months after PEA, yearly for the following 5 years, and then at 7, 10, and 15 years postoperatively. Both early and late survivals were excellent. Survival rate at 3 months, 1 year, and 3 years were respectively of 89.5±2.7%, 87.3±3.0%, and 82.7±3.6%. Survival rates had not changed at 5, 7, and 10 years postoperative. Three months after PEA, 29 (58%) subjects were within NYHA class I, 18 (36%) in class II, and 3 (6%) in class III. At 1-year follow-up, 40 (80%) patients were within NYHA class I, 10 (20%) in class II. A statistically significant difference exists not only between the preoperative and the postoperative data (p <0.0001), but also between the functional status at 3 months and the other two postoperative controls (p <0.001). Table summarizes the results of hemodynamic tests collected at three months, one year and three years on the first 35 patients who completed the follow-up program.
Hemodynamic data from 35 patients participating to the Pavia Pulmonary Endarterectomy Program with complete 3-year follow-up. CVP mPAP CO CI PVR PVRI CVP (mmHg) central venous pressure; mPAP (mmHg) mean pulmonary artery pressure; CO (L/min) cardiac output; CI (L/min/m2) cardiac index; PVR (dynes/sec/cm-5) pulmonary vascular resistances; PVRI (dynes/sec/cm-5/m2) pulmonary vascular resistances index; RV-EF (%) right ventricle ejection fraction. RV-EF A: Before-PEA 7±6 48±12 3.3±0.9 1.8±0.5 1125±412 2027±731 15±8 B:Before discharge 5±4 25±10 5.2±1.1 2.9±0.5 289±142 505±234 32±8 C: 3 months 2±2 24±11 5.1±1.4 2.8±0.6 231±198 542±271 32±7 D: 1 year 1±2 23±12 5.0±1.1 2.7±0.6 290±191 531±343 35±8 E: 3 years 2±2 24±12 4.9±1.1 2.6±0.5 317±226 579±393 34±8 p value A vs. B: nsA vs. C, D, and E: <0.0001B vs. C, D and E: <0.05 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E: <0.0001 A vs. B, C, D and E:
Echocardiographic evaluation of systolic and mean pulmonary artery pressure in the follow-up of patients with pulmonary hypertension