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ObjectivePregnancy in women with aortic coarctation (CoA) has an estimated moderately increased risk (mWHO II–III) of adverse cardiovascular, obstetric or fetal events, but prospective data to validate this risk classification are scarce. We examined pregnancy outcomes and identified associations with adverse outcomes.MethodsPregnancies in women with CoA were selected from the worldwide prospective Registry of Pregnancy and Cardiac Disease (ROPAC, n=303 out of 5739), part of the European Society of Cardiology EURObservational Research Programme. The frequency of and associations with major adverse cardiac events (MACE) and hypertensive disorders (pregnancy-induced hypertension, (pre-)eclampsia or haemolysis, elevated liver enzymes and low platelets syndrome) were analysed.ResultsOf 303 pregnancies (mean age 30 years, pregnancy duration 39 weeks), 9.6% involved unrepaired CoA and 27.1% were in women with pre-existing hypertension. No maternal deaths or aortic dissections occurred. MACE occurred in 13 pregnancies (4.3%), of which 10 cases were of heart failure (3.3%). Univariable associations with MACE included prepregnancy clinical signs of heart failure (OR 31.8, 95% CI 6.8 to 147.7), left ventricular ejection fraction 1 (OR 11.4, 95% CI 3.6 to 36.3) and cardiac medication use (OR 4.9, 95% CI 1.3 to 18.3). Hypertensive disorders of pregnancy occurred in 16 (5.3%), cardiac medication use being their only predictor (OR 3.2, 95% CI 1.1 to 9.6). Premature births were 9.1%, caesarean section was performed in 49.7% of pregnancies. Of 4 neonatal deaths, 3 were after spontaneous extreme preterm birth.ConclusionsThe ROPAC data show low MACE and hypertensive disorder rates during pregnancy in women with CoA, suggesting pregnancy to be more safe and better tolerated than previously appreciated.

OBJECTIVE High altitude-related hypoxia induces pulmonary vasoconstriction. In Fontan patients without a contractile subpulmonary ventricle, an increase in pulmonary artery pressure is expected to decrease circulatory output and reduce exercise capacity. This study investigates the direct effects of short-term high altitude exposure on pulmonary blood flow (PBF) and exercise capacity in Fontan patients. METHODS 16 adult Fontan patients (mean age 28±7 years, 56% female) and 14 matched controls underwent cardiopulmonary exercise testing with measurement of PBF with a gas rebreathing system at 540 m (low altitude) and at 3454 m (high altitude) within 12 weeks. RESULTS PBF at rest and at exercise was higher in controls than in Fontan patients, both at low and high altitude. PBF increased twofold in Fontan patients and 2.8-fold in the control group during submaximal exercise, with no significant difference between low and high altitude (p=0.290). A reduction in peak oxygen uptake at high compared with low altitude was observed in Fontan patients (22.8±5.1 and 20.5±3.8 mL/min/kg, p

In patients with heart failure from acquired cardiomyopathy, respiratory and skeletal muscle weakness is common and is an independent predictor for adverse events. Despite a different underlying pathology, many young adults with congenital heart disease (CHD) develop a syndrome comparable to heart failure from acquired cardiomyopathy and may be at risk for a similar skeletal muscle weakness.To assess respiratory and skeletal muscle strength in adults with complex CHD.Respiratory and skeletal muscle function was assessed in 51 adults; 41 with complex CHD (16 tetralogy of Fallot, 11 univentricular anatomy with Fontan operation and 14 with subaortic right ventricles) and 10 controls. Maximal inspiratory (MIPs) and expiratory (MEPs) pressures, handgrip strength, lung volumes and aerobic capacity (peak VO₂) were measured. Results In patients with CHD (age 34 ± 13 years), average% predicted MIPs, MEPs and handgrip strength were lower than in controls (77 ± 27% vs 106 ± 28%, 85 ± 32% vs 116 ± 41% and 72 ± 15% vs 93 ± 14%, respectively, p ≤ 0.01). There was no significant difference in muscle weakness between CHD subgroups. In 39% of patients with CHD, the handgrip strength, and in 22%, respiratory muscle strength was70% predicted. These patients had a significantly lower peak VO₂ (50 ± 12% vs 64 ± 14% predicted, p=0.008).Respiratory and skeletal muscle weakness is common in young adults with complex CHD and similar to that found in older adults with advanced heart failure from acquired heart disease.