warfarin and Eisenmenger-Complex

We present a 36-year-old male patient with a previous diagnosis (22 years) of Eisenmenger's syndrome, who had a giant proximal pulmonary artery aneurysm complicated by massive thrombus formation. The patient experienced paroxysmal atrial fibrillation attacks for the past month. His functional capacity was New York Heart Association class III. Chest radiography showed aneurysmal dilatation in the left pulmonary artery. The patient was assessed by transthoracic echocardiography and multislice computed tomography. There was mild narrowing in the thick and calcified pulmonary valve (peak systolic gradient 35 mmHg) and moderate regurgitation. The mean pulmonary artery pressure was estimated as 50 mmHg. The diameters of the main, left, and right pulmonary arteries were 6.5 cm, 10 cm, and 3.7 cm, respectively. There was a massive thrombus in the aneurysmatic left pulmonary artery. The patient was referred to the cardiovascular surgery department for pulmonary artery reconstruction and cardiopulmonary transplantation. In addition, medical treatment was instituted with warfarin for thrombus and paroxysmal atrial fibrillation, metoprolol for atrial fibrillation, and bosentan for pulmonary hypertension. The patient's functional capacity showed improvement after the first month of medical treatment and no complications were seen within a year follow-up.

Topics: Anti-Arrhythmia Agents; Anticoagulants; Atrial Fibrillation; Calcinosis; Child; Coronary Vessel Anomalies; Eisenmenger Complex; Humans; Male; Metoprolol; Pulmonary Embolism; Radiography, Thoracic; Tomography, X-Ray Computed; Treatment Outcome; Warfarin

Endothelial dysfunction has been reported in hypoxaemic patients with the Eisenmenger syndrome, but a direct correlation between levels of endothelial markers and the severity of hypoxaemia has not been explored. With this in mind, we compared the levels in the plasma of tissue-type plasminogen activator, thrombomodulin, and von Willebrand factor in 25 patients with the Eisenmenger syndrome. They had a median age of 31 years, and were divided into 2 groups according to their recent clinical history. Thus, 18 patients were stable, being in functional class II or III, seen as outpatients, and having peripheral saturations of oxygen of 89 plus or minus 5 percent. In contrast, 7 patients were unstable, showing episodes of symptoms placing them in functional class IV, requiring care in hospital, and manifesting saturations of oxygen of 77 plus or minus 5 percent. We were able to follow 12 patients, 8 who were stable and 4 unstable, for 24 months. At baseline, levels of von Willebrand factor were higher in the unstable patients when compared to those who were stable, at 142 plus or minus 29 and 110 plus or minus 25 units per decilitre, respectively (p equal to 0.013). This correlated positively with oxygen desaturation (p less than 0.020). The structural abnormalities also correlated positively with the magnitude of hypoxaemia (p less than 0.020). Levels remained higher in the unstable patients throughout the period of follow-up (p equal to 0.006). Tissue-type plasminogen activator was also increased, at 14.3 plus or minus 8.4 versus 6.5 plus or minus 2.7 nanograms per millilitre in controls (p less than 0.001), whereas thrombomodulin was decreased, with values of 14.4 versus 34.6 nanograms per millilitre in controls (p for median values of less than 0.001). There was no correlation with saturations of oxygen. We conclude that measurement of von Willebrand factor, as compared with tissue-type plasminogen activator and thrombomodulin, will prove a better marker of endothelial response to hypoxaemia in patients with the Eisenmenger syndrome.

Topics: Adolescent; Adult; Biomarkers; Case-Control Studies; Child; Child, Preschool; Eisenmenger Complex; Endothelium, Vascular; Humans; Hypoxia; Male; Middle Aged; Oxygen; Thrombomodulin; Tissue Plasminogen Activator; von Willebrand Factor; Warfarin

This is the report of a series of eight patients with pulmonary hypertension (primary and secondary) who delivered at the McMaster University Medical Centre between 1978 and 1987. Seven of the eight patients delivered vaginally and had a successful outcome. The eighth patient was admitted as an emergency and died shortly after Caesarean section under general anaesthesia, performed to save the infant. The other seven patients were all managed by a team, including anaesthetists, cardiologists and obstetricians, from about 25 wk. The patients were hospitalized pre-partum and received oxygen therapy and anticoagulation with heparin. Analgesia in labour was managed, once anticoagulation was reversed, by low concentrations of epidural bupivacaine (0.125%-0.375%) and fentanyl. The patients were monitored during labour and delivery with oximetry and arterial and central venous pressure lines. Pulmonary arterial lines were not used because of increased risk and questionable usefulness. Vaginal delivery was managed with vacuum extraction or forceps lift-out to minimize the stress of pushing. After delivery, all patients were monitored in an intensive care unit for several days, anticoagulation was restarted, and all patients were discharged home taking oral anticoagulant therapy. The successful management of pulmonary hypertension in pregnancy should include team management started early in pregnancy and controlled vaginal delivery utilizing epidural analgesia.

Topics: Adult; Anesthesia, Epidural; Anesthesia, Obstetrical; Delivery, Obstetric; Ductus Arteriosus, Patent; Eisenmenger Complex; Female; Heart Septal Defects, Atrial; Heart Septal Defects, Ventricular; Heart Valve Diseases; Heparin; Humans; Hypertension, Pulmonary; Mitral Valve; Monitoring, Physiologic; Obstetric Labor Complications; Pregnancy; Pregnancy Complications, Cardiovascular; Pregnancy Outcome; Warfarin