Pregnancy and Successful Delivery in a Patient with Chronic Thromboembolic Pulmonary Hypertension (CTEPH) Previously Submitted to Pulmonary Endarterectomy (PEA)

Abstract 4329

Chronic thromboembolic pulmonary hypertension (CTEPH) 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. CTEPH is a condition that is recognised in an increased percentage of patients. Pulmonary endarterectomy (PEA) is recognized as being the only curative option for a subgroup of those patients, but anaesthesiologists and intensivists face many challenges in how they manage these patients perioperatively. Ultimately, it is the combination of skills in a multidisciplinary team that leads to a successful procedure and dramatically improves patient's quality of life and life expectancy. Careful pre- and post-operative management is therefore essential for such a successful outcome following PEA. In 1994 in Pavia was started 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. Pregnancy in women with pulmonary hypertension (PH) is reported to carry a maternal mortality rate of 30–56%. No report is available on the management of pregnancy and delivery in patients with CTPH. We report our experience of the management of a pregnancy in a patient previously submitted to PEA in whom pressures decreased significantly but remained higher than normal due to partial intervention. EFS, 29 yrs, had first hemoptysis in 2001. Since then, she suffered effort dyspnea. A second hemoptysis occurred in 2004. She was admitted to hospital as having “Multiple foci pneumonia, with pulmonary hypertension of unclear origin”. In 2005 patient was admitted to a different hospital. A CT scan showed: “Congenital right pulmonary artery agenesia associated with bilateral multiple artero-venous malformations. NYHA Class III”. Patients was transferred to our hospital. Thrombophilic workout resulted negative. PaO₂: 79.9 mmHg, pulmonary artery pressures (PAP): 130/60/13 mmHg, pulmonary vascular resistances (PVR): 1.083 dynes/sec/min⁻⁵. CT angio-scan: Severe dilation of the common pulmonary artery (34 mm). Right pulmonary artery visible only at proximal level. Bronchial artery dilation, bilaterally. V/Q scan: Absent visualization of the right pulmonary artery. Perfusion absent. Arteriography: Clearcut thrombosis of the right pulmonary artery (initial tract). Multiple typical CTPH lesions of the left pulmonary artery. Lower limb compression ultrasound (CUS): No sign of deep or superficial vein thrombosis. On 11 april, 2005 PEA was performed on the left side, with an attempt on the right side. No agenesia of the right pulmonary artery was found. Probable occlusion in early age, with evolution in fibrosis. Post surgery, PaO₂: 94.3 mmHg, PAP: 53/32/15 mmHg, PVR: 453 dynes/sec/min⁻⁵. On September 16, 2005 she was pregnant, seventh week. After careful multidisciplinary counseling, patient decided to continue pregnancy. Anticoagulant treatment was switched from warfarin to low molecular weight heparin (LMWH), therapeutic dosage. Two days before elective delivery, LMWH was reduced to prophylactic dosages. On march 2, 2006 after an uncomplicated Caesarean section under general anesthesia, she delivered a healthy baby girl. During the following months, PAPs and functional parameters normalized. Patient is today in relatively good health. Is under oral vitamin K antagonists treatment. In 2007, being pregnant again, opted for therapeutic abortion. Maternal mortality in parturients with PAH or CTPH remains prohibitively high, despite lower death rates than previous decades. Early advice on pregnancy risks, including contraception, remains paramount. Women with PAH or CTPH who become pregnant warrant a multidisciplinary approach with consideration of appropriate therapies.