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BACKGROUND: Eisenmenger syndrome (ES) precipitates the extreme manifestation of pulmonary hypertension, which leads to severe functional limitation and poor quality of life. The propose of the current study was: 1) examined the acute effects of 40% oxygen supplementation during the 6-minute walk test (6MWT); and 2) evaluate the relation between exercise capacity and clinical cardiac parameters in patients with ES. METHODS: Thirty subjects were prospectively included; all were submitted to a 6MWT with compressed air and with 40% of oxygen. Heart rate recovery at the first minute (HRR1) and perceived effort Borg scale for dyspnea and lower limb fatigue were recorded in both tests scenarios. RESULTS: The 6MWT distance was modestly, negatively associated with pulmonary vascular resistance (PVR) [r=0.46, p=0.02]. Patients improved 6MWT distance (p<0.001) and exhibited a faster HRR1 (p<0.001) with 40% supplemental oxygen compared to compressed air. With 40% supplemental oxygen, subjects revealed lower dyspnea and lower limb fatigue compared to 6MWT without oxygen supplementation (p<0.001). The amount of change in the 6MWT distance from air to oxygen was moderate, positively associated with tricuspid annular plane systolic excursion (TAPSE) and right ventricular fractional area change (RVFAC) [r=0.50, p=0.03; r=0.64, p<0.001, respectively]. CONCLUSION: Acute 40% oxygen supplementation in patients with Eisenmenger syndrome led to an improvement in 6MWT distance, faster HRR1 and lower dyspnea and lower limb fatigue perception. Moreover, functional capacity was positively associated with right ventricular parameters.

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Eisenmenger syndrome is very rare in pregnant women. Debates remain concerning the management of Eisenmenger syndrome in this patient population and the prognosis is unclear in terms of maternal and fetoneonatal outcomes. Epidural analgesia is preferred for Cesarean section as it alleviates perioperative pain and reduces the pulmonary and systemic vascular resistances. Maternal mortality in the presence of Eisenmenger syndrome is reported as 30-50% and even up to 65% in those with Cesarean section. The major causes of death could be hypovolemia, thromboembolism and preeclampsia. Pregnancy should ideally be avoided in a woman with Eisenmenger syndrome concerning the high maternal mortality rate and probable poor prognosis of the baby. A short labour and an atraumatic delivery under epidural block are preferred in the women with a strong desire of pregnancy. The purpose of this article is to discuss the debates of Eisenmenger syndrome in pregnancy and the possible resolutions.

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Abstract Eisenmenger syndrome is very rare in pregnant women. Debates remain concerning the management of Eisenmenger syndrome in this patient population and the prognosis is unclear in terms of maternal and fetoneonatal outcomes. Epidural analgesia is preferred for Cesarean section as it alleviates perioperative pain and reduces the pulmonary and systemic vascular resistances. Maternal mortality in the presence of Eisenmenger syndrome is reported as 30-50% and even up to 65% in those with Cesarean section. The major causes of death could be hypovolemia, thromboembolism and preeclampsia. Pregnancy should ideally be avoided in a woman with Eisenmenger syndrome concerning the high maternal mortality rate and probable poor prognosis of the baby. A short labour and an atraumatic delivery under epidural block are preferred in the women with a strong desire of pregnancy. The purpose of this article is to discuss the debates of Eisenmenger syndrome in pregnancy and the possible resolutions.

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UNLABELLED: Obesity and Eisenmenger's syndrome are entities widely studied. However, its association is unusual and has not been reported. A wide range of gas exchange abnormalities have been describe in both groups. In the severe obese patients this abnormalities are attributed to a ventilation/perfusion mismatch and to an increase pulmonary venous-arterial shunt, that correlates with the lung volume. In severe obese patients with the Eisenmenger's syndrome, this correlation is unknown. METHODS: We studied 28 obese subjects paired by body mass index > 30 kg/m2. Assigned to two groups, obese with Eisenmenger's syndrome and obese without the syndrome. Clinical variables, respiratory function, echocardiography and gas exchange pre and post-deep breathing maneuver were obtained. STATISTICAL ANALYSIS: The variables are expressed with mean+/-standard deviation. Student t test for paired groups and Pearson correlation coefficient were gathered for the differences and associations between groups. A p-value <0.05 was considered significant. RESULT: Age was 48.57 +/- 10.32 vs 60.86 +/- 10.47 y.o. respectively, p < 0.004. Systolic pulmonary arterial pressure 104.36 +/- 37 vs 50.1 +/- 12 mm Hg, p < 0.001. The arterial oxygen pressure at rest and during the deep breathing maneuver in each group was: 51.64 +/- 6.38 vs 57.14 +/- 11, p < 0.188 and 56.29 +/- 11.15 vs 72 +/- 11.83, p < 0.001; venous-arterial shunt 12.79 +/- 3.66 vs 13.07 +/- 4.84, p < 0.767 and 9.21 +/- 3.77 vs 6.5 +/- 2.28, p < 0.001; alveolar arterial oxygen difference 271.14 +/- 79.92 vs 243.79 +/- 92.07, p < 0.001, respectively. CONCLUSION: Obese subjects with Eisenmenger's syndrome, did not have significant improvement of gas exchange with the deep breathing maneuver.

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Preliminary studies on sleep of patients with congenital heart disease and Eisenmenger's syndrome (ES) at our institution demonstrated nocturnal worsening arterial unsaturation, which appeared to be a body position-related phenomenon. To investigate the potential effect of body position on gas exchange in ES, we carried out a prospective study of 28 patients (mean age, 34.8 +/- 11.7 yr) with established ES due to congenital heart disease. In every patient, arterial blood gases were performed during both sitting and supine positions under three different conditions: room air, while breathing 100% oxygen, and after breathing oxygen at a flow rate of 3 L/min through nasal prongs. Alveolar oxygen pressure (PaO2) for the calculation of alveolar-arterial oxygen tension differences (AaPO2) was derived from the alveolar gas equation using PaCO2 and assuming R = 1. We used paired t test, repeated-measures two-way ANOVA with Bonferroni's test, and regression analysis. From sitting to supine position on room air, there was a significant decrease in PaO2 (from 52.5 +/- 7.5 to 47.5 +/- 5.5 mm Hg; p < 0. 001) and SaO2 (from 86.7 +/- 4.6 to 83.3 +/- 4.9%; p < 0.001), both of which were corrected by nasal O2 (to 68.2 +/- 21 mm Hg and to 92 +/- 4%, respectively, p < 0.005). PaCO2 and pH remained unchanged. The magnitude of the change in PaO2 correlated with the change in AaPO2 on room air (r = 0.77; p < 0.01) but not with the change in AaPO2 on 100% oxygen. It is concluded that in adult patients with ES there is a significant decrease in PaO2 and SaO2 when they change from the sitting to the supine position. A ventilation-perfusion (V/Q) distribution abnormality and/or a diffusion limitation phenomenon rather than an increase in true shunt may be the mechanisms responsible for this finding. The response to nasal O 2 we observed warrants a trial with long-term nocturnal oxygen therapy in these patients.

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Interruption of pregnancy is usually recommended for pregnant women with Eisenmenger's syndrome. We studied 13 pregnancies in 12 women with this syndrome, who decided to carry on with their pregnancy despite recommendation for therapeutic abortion. The mean age was 27 years. Five patients had ventricular septal defect; two, persistent ductus arteriosus; one, a combination of both; two, atrial septal defect; one, atrioventricular septal defect and one patient a combination of ventricular and atrial septal defects. Mean systolic and diastolic arterial pulmonary pressures were 112.7 and 61.7, mmHg, respectively. There were three spontaneous abortions, one premature labour at 23 weeks of gestation and two maternal deaths during the 23 and 27 weeks of gestation. Seven patients who reached the end of the second trimester were hospitalized until delivery and received heparin (20,000 to 40,000 units per day) and oxygen therapy. Caesarean section was performed in all patients as a result of worsening maternal or fetal clinical condition during the third trimester of gestation. all the mothers were discharged from hospital but one of them died on the 30th day post-partum. Five of the eight infants were premature, three were small babies for gestational age and all were discharged from hospital with the exception of one who died 48 h after birth. In conclusion, although pregnancy should be discouraged in women with Eisenmenger's syndrome, it can be successful. In this study, prolonged bed rest, the use of heparin and oxygen therapy presumably positively influenced maternal and infant outcomes.

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BACKGROUND: Eisenmenger's syndrome remains one of the greatest challenges in lung transplantation. METHODS AND RESULTS: Since October 1990, seven such patients with Eisenmenger's syndrome received isolated pulmonary grafts (six double lungs and one single lung). Mean patient age was 32 +/- 6 years (two men and five women). The preoperative mean pulmonary arterial pressure was 90.7 +/- 31.2 mm Hg, and the ventriculoscintigram showed markedly enlarged right ventricle and normal left ventricular function with ejection fraction of 0.660 +/- 0.115. Three atrial septal defects and four patent ducti arteriosus were repaired concomitantly. Excised lung histology showed plexogenic pulmonary arteriopathy with Heath-Edwards' grade 4 through 6. One double lung patient who had preexisting systemic vascular collapse died intraoperatively. The other six patients tolerated transplantation, and on the first operative day, mean pulmonary artery pressure decreased to 22.4 +/- 7.3 mm Hg (P < .002) and gas exchange was acceptable with an arterial/alveolar oxygen tension ratio of 0.47 +/- 0.15. Two patients died of mediastinal and pulmonary infection. The follow-up for the four survivors ranged from 13 to 25 months after transplantation. CONCLUSIONS: Our preliminary experience shows that concomitant isolated lung transplantation with cardiac repair could be a viable therapeutic option for patients with Eisenmenger's syndrome and normal left ventricular function. Dynamic right ventricular outflow obstruction is a potential hemodynamic problem in these pulmonary recipients.

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