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Adults with systemic right ventricle have a significant risk for long-term complications such as arrhythmias or heart failure.
A nationwide retrospective study based on the German National Register for Congenital Heart Disease was performed. Patients with transposition of the great arteries after atrial switch operation or congenitally corrected TGA were included.
Two hundred and eight-five patients with transposition of the great arteries after atrial switch operation and 95 patients with congenitally corrected transposition of the great arteries were included (mean age 33 years). Systolic function of the systemic ventricle was moderately or severely reduced in 25.5 % after atrial switch operation and in 35.1% in patients with congenitally corrected transposition. Regurgitation of the systemic atrioventricular valve was present in 39.5% and 43.2% of the cases, respectively. A significant percentage of patients also had a history for supraventricular or ventricular arrhythmias. However, polypharmacy of cardiovascular drugs was rare (4.5%) and 38.5 % of the patients did not take any cardiovascular medication. The amount of cardiovascular drugs taken was associated with NYHA class as well as systemic right ventricular dysfunction. Patients with congenitally corrected transposition were more likely to receive pharmacological treatment than patients after atrial switch operation.
A significant portion of patients with systemic right ventricle suffer from a relevant systemic ventricular dysfunction, systemic atrioventricular valve regurgitation, and arrhythmias. Despite this, medication for heart failure treatment is not universally used in this cohort. This emphasises the need for randomised trials in patient with systemic right ventricle.
Most patients born with CHD nowadays reach adulthood, and thus quality of life, life situation, and state of medical care aspects are gaining importance in the current era. The present study aimed to investigate whether patients’ assessment depends on their means of occupation. The findings are expected to be helpful in optimising care and for developing individual treatment plans.
The present study was based on an online survey conducted in cooperation with patient organisations. Participants were recruited from the database of the German National Register for Congenital Heart Defects. In total, 1828 individuals (777 males, 1051 females) took part. Participants were asked to rate aspects such their state of health on a six-tier scale (1=worst specification). Response behaviour was measured against the background of occupational details.
Training for or pursuing a profession was found to be significantly associated with participants’ rating of five of the six examined aspects (p<0.05). Sex seemed to play an important part in four of the six aspects.
An optimal treatment plan for adults with CHD should always consider aspects such as sex and employment status. To work out such an optimal and individual treatment plan for each adult CHD patient, an objective tool to measure patients’ actual CHD-specific knowledge precluding socially accepted response bias would be very useful.
Inappropriate heart rate response to exercise – chronotropic incompetence – and exercise intolerance are common in patients with a systemic right ventricle. We aimed to assess the relationship between heart rate increase, oxygen consumption, and timing of the right ventricular cardiac cycle in this cohort.
We prospectively studied nine patients with systemic right ventricles and pre-existing pacemakers using Doppler-echocardiography and treadmill exercise testing. Echocardiography was performed at increasing heart rates. Exercise tests were performed with baseline pacemaker settings and with optimised heart rate response in random order. In addition, eight age- and gender-matched controls underwent exercise testing using a similar exercise protocol.
Patients with a systemic right ventricle had significantly lower peak oxygen consumption compared to controls – 12.6 plus or minus 6.8 versus 31.4 plus or minus 6.6 metres per kilogram per minute (p = 0.0006) – at baseline and active pacemaker reprogramming failed to increase peak oxygen consumption in this cohort – 12.6 plus or minus 6.8 versus 12.4 plus or minus 4.9 millilitres per kilogram per minute (p = NS) at baseline and with reprogramming, respectively. We found not only a marked increase in total isovolumic time but also a significant reduction in total filling time and the aortic velocity time integral, p-value is less than 0.001 for all, at higher heart rates compared to baseline conditions.
This study suggests that despite chronotropic incompetence at baseline, rate-responsive pacing does not improve exercise capacity in patients with a systemic right ventricle. It further indicates that high heart rates may be detrimental in these patients by reducing diastolic filling and stroke volume. These findings may have clinical implications when considering implantation of a permanent pacemaker in this cohort.
Atrial septostomy is performed in patients with severe pulmonary arterial hypertension, and has been shown to improve symptoms, quality of life and survival. Despite recognized clinical benefits, the underlying pathophysiologic mechanisms are poorly understood. We aimed to assess the effects of right-to-left shunting on arterial delivery of oxygen, mixed venous content of oxygen, and systemic cardiac output in patients with pulmonary arterial hypertension and a fixed flow of blood to the lungs. We formulated equations defining the mandatory relationship between physiologic variables and delivery of oxygen in patients with right-to-left shunting. Using calculus and computer modelling, we considered the simultaneous effects of right-to-left shunting on physiologies with different pulmonary flows, total metabolic rates, and capacities for carrying oxygen. Our study indicates that, when the flow of blood to the lungs is fixed, increasing right-to-left shunting improves systemic cardiac output, arterial blood pressure, and arterial delivery of oxygen. In contrast, the mixed venous content of oxygen, which mirrors the average state of tissue oxygenation, remains unchanged. Our model suggests that increasing the volume of right-to-left shunting cannot compensate for right ventricular failure. Atrial septostomy in the setting of pulmonary arterial hypertension, therefore, increases the arterial delivery of oxygen, but the mixed systemic saturation of oxygen, arguably the most important index of tissue oxygenation, stays constant. Our data suggest that the clinically observed beneficial effects of atrial septostomy are the result of improved flow of blood rather than augmented tissue oxygenation, provided that right ventricular function is adequate.
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