Novel strategies for the management of right ventricular failure with pulmonary hypertension.

The authors report a case where four novel strategies were successfully used in the management of advanced right ventricular failure with severe pulmonary hypertension and complex adult congenital heart disease. These included combination and optimisation of three advanced therapies for pulmonary hypertension, therapeutic abdominal paracentesis, correction of underlying metabolic/endocrine disturbance and a new inotropic agent.

Reference values for exercise limitations among adults with congenital heart disease. Relation to activities of daily life--single centre experience and review of published data.

Aims: We aimed to investigate the distribution of exercise capacity across the spectrum of adult congenital heart disease (ACHD) using own data and the published experience and to provide diagnosis, gender-, and age- specific reference values.

Methods And Results: Publications describing exercise capacity in ACHD patients using cardiopulmonary exercise testing (CPET) were identified (n = 2286 patients in 23 papers). In addition, we included 2129 patients who underwent CPET at our own institution.

Total isovolumic time relates to exercise capacity in patients with transposition of the great arteries late after atrial switch procedures.

Background: Systemic right ventricular systolic dysfunction is common late after atrial switch surgery for transposition of the great arteries. Total isovolumic time is the time that the ventricle is neither ejecting nor filling and is calculated without relying on geometric assumptions. We assessed resting total isovolumic time in this population and its relationship to exercise capacity.

Detrimental impact of socioeconomic status on exercise capacity in adults with congenital heart disease.

Objectives: To evaluate the relationship between socioeconomic status (SES), access to physical activity resources, urban-rural dwelling, levels of pollution and exercise capacity in adult congenital heart disease (ACHD) patients.

Background: Exercise intolerance is prevalent in ACHD and the contributing factors are poorly understood.

Methods: A total of 1268 ACHD patients living in England who underwent cardiopulmonary exercise testing at our center were included.

Peak oxygen uptake correlates with disease severity and predicts outcome in adult patients with Ebstein's anomaly of the tricuspid valve.

Background: Ebstein's anomaly of the tricuspid valve often results in biventricular dysfunction and functional deterioration. Little is known about the relation between exercise capacity, disease severity and outcome in adults with Ebstein's anomaly.

Methods: Data on all patients with Ebstein's anomaly of the tricuspid valve who underwent cardiopulmonary exercise testing in our tertiary center were collected.

Atrial septostomy in patients with pulmonary hypertension: should it be recommended?

Treatment options for patients with advanced pulmonary hypertension (PH) are limited. Iatrogenic creation of an interatrial communication (i.e.

Predictors of morbidity and mortality in contemporary Fontan patients: results from a multicenter study including cardiopulmonary exercise testing in 321 patients.

Aims: previous studies have established an association between exercise intolerance and increased morbidity and mortality in congenital heart disease patients. We aimed to clarify if exercise intolerance is associated with poor outcome in Fontan patients and to identify risk factors for mortality, transplantation, and cardiac-related hospitalization.

Methods And Results: a total of 321 Fontan patients (57% male, mean age 20.

The right heart and pulmonary circulation (IX). The right heart in adults with congenital heart disease.

Right ventricular dysfunction is not uncommon in adults with congenital heart disease. In congenital heart disease, unlike acquired heart disease, the right ventricle is not always the subpulmonary ventricle: it may support the systemic circulation as it does in patients with transposition complexes. The result is chronic right ventricular pressure overload.

Replacement therapy for iron deficiency improves exercise capacity and quality of life in patients with cyanotic congenital heart disease and/or the Eisenmenger syndrome.

Introduction: Iron deficiency is common in cyanotic congenital heart disease (CHD) and results in reduced exercise tolerance. Currently, iron replacement is advocated with limited evidence in cyanotic CHD. We investigated the safety and efficacy of iron replacement therapy in this population.

Quality of life and functional capacity can be improved in patients with Eisenmenger syndrome with oral sildenafil therapy.

Background: Patients with Eisenmenger syndrome (ES) have a decreased exercise capacity and poor quality of life (QoL). While patients may survive to middle adulthood, the burden of disease is disabling. Sildenafil seems to improve exercise tolerance and hemodynamics, but there is no data to date on its impact on QoL.

Usefulness of natriuretic Peptide levels to predict mortality in adults with congenital heart disease.

Neurohormonal activation is prevalent in adults with congenital heart disease, but its relation to outcome remains unknown. B-type natriuretic peptide (BNP) and atrial natriuretic peptide (ANP) were measured prospectively in 49 patients with adult congenital heart disease, who were followed up for a median of 7.9 years (interquartile range 7.

Improved survival among patients with Eisenmenger syndrome receiving advanced therapy for pulmonary arterial hypertension.

Background: Advanced therapy (AT) for pulmonary arterial hypertension in the context of congenital heart disease (Eisenmenger syndrome) improves pulmonary hemodynamics, functional class, and the 6-minute walk test. We examined the potential effect of AT on survival in this population.

Methods And Results: Data on all Eisenmenger patients attending our center over the past decade were collected.

Hyponatraemia: A strong predictor of mortality in adults with congenital heart disease.

Aims: We studied the prevalence of hyponatraemia and its prognostic implications in a large population of adult patients with congenital heart disease (ACHD).

Methods And Results: A total of 1004 ACHD patients were retrospectively entered in this study (mean age 36.2 +/- 14.

Anemia in adults with congenital heart disease relates to adverse outcome.

Objectives: To assess the relation of anemia in noncyanotic adults with congenital heart disease (ACHD) to functional capacity and mortality.

Background: Anemia is common in acquired heart failure and affects prognosis. The presence of anemia and its relation to outcome in ACHD remain unknown.

Exercise intolerance in patients with congenitally corrected transposition of the great arteries relates to right ventricular filling pressures.

Background: Patients with congenitally corrected transposition of the great arteries (ccTGA) have significantly reduced exercise tolerance. Progressive right ventricular (RV) dysfunction with tricuspid regurgitation (TR) and other haemodynamic lesions are common among them. We hypothesised that interaction of these factors may result in increased systemic RV filling pressure, which in turn impact on exercise capacity.

Burden of coronary artery disease in adults with congenital heart disease and its relation to congenital and traditional heart risk factors.

As adult patients with congenital heart disease (CHD) grow older, the risk of developing coronary artery disease (CAD) increases. We sought to estimate the prevalence of CAD in adult patients with CHD, the safety of coronary angiography in this setting, and the potential relation of CAD to clinical and hemodynamic parameters. Two hundred fifty adult patients with CHD (mean age 51 +/- 15 years; 53% men) underwent selective coronary angiography in our center for reasons other than suspected CAD.

Has there been any progress made on pregnancy outcomes among women with pulmonary arterial hypertension?

Pregnancy in women with pulmonary arterial hypertension (PAH) is considered to be associated with prohibitive maternal mortality. During the past decade, new advanced therapies for PAH have emerged and progress in high-risk pregnancy management has been made. We examined whether these changes have improved outcomes in parturients with PAH.

Pulmonary arterial hypertension in adults with congenital heart disease: distinct differences from other causes of pulmonary arterial hypertension and management implications.

Purpose Of Review: To present the available data on pathophysiology, clinical presentation, prognosis, and especially management strategies for adult patients with congenital heart disease (CHD) and pulmonary arterial hypertension (PAH). Particular emphasis is placed on differences between other types of PAH and CHD-related PAH, in which clinical presentation and management relate to a constellation of factors, both pulmonary and cardiac.

Recent Findings: Pulmonary vascular disease in adults with CHD and especially its extreme expression, the Eisenmenger syndrome, is a chronic disease with slow progression, leading to multiorgan failure and death, decades after its first clinical presentation.

Circulating endothelial progenitor cells in patients with Eisenmenger syndrome and idiopathic pulmonary arterial hypertension.

Background: Impaired endothelial homeostasis underlies the pathophysiology of pulmonary arterial hypertension (PAH). We speculated that PAH patients are deficient in circulating endothelial progenitor cells (EPCs), potentially contributing to endothelial dysfunction and disease progression.

Methods And Results: We recruited 41 patients with Eisenmenger syndrome (13 with Down syndrome), 55 with idiopathic PAH, and 47 healthy control subjects.

Evaluating operability in adults with congenital heart disease and the role of pretreatment with targeted pulmonary arterial hypertension therapy.

Pulmonary arterial hypertension (PAH) associated with congenital heart disease remains a major problem despite advances in cardiac surgery. Recently, advanced therapies for PAH have become available and have been effective in reducing pulmonary vascular resistance and symptoms in patients with near-systemic pulmonary arterial pressures, previously thought to have irreversible pulmonary vascular disease. This has led to a new dilemma, namely could intracardiac communications previously considered inoperable due to severe pulmonary vascular disease become amenable to surgery after successful treatment with advanced therapy? We address, hereby, the potential merits and hazards of a "treat-and-repair" approach using advanced therapies in patients with PAH associated with congenital heart disease.

Bronchial artery embolization for pulmonary arterial hypertension and recurrent hemoptysis?

Embolization of bronchial collaterals has been suggested as a means of controlling the recurrence of hemoptysis in patients with pulmonary arterial hypertension. In conclusion, the investigators present a patient in which this procedure failed to prevent further episodes of hemoptysis and discuss whether this procedure should be offered routinely, given the lack of sufficient data to support this approach.

Deep vein thrombosis in a patient with congenital heart disease and permanent transfemoral pacing.

A 45-year-old woman with previous repair of coarctation of aorta, ventricular septal defect closure, and progressive decline in her exercise capacity was admitted for the treatment of left leg deep venous thrombosis (DVT). She had a history of complete heart block and insertion of a pectoral pacemaker. After numerous problems with pocket infections, multiple box changes and fracture of the atrial lead, the pectoral system was extracted, and a transfemoral permanent pacemaker was implanted.

Long-term safety, tolerability and efficacy of bosentan in adults with pulmonary arterial hypertension associated with congenital heart disease.

Objective: To examine long-term safety and efficacy of bosentan--an oral dual endothelin receptor antagonist--in patients with pulmonary hypertension associated with congenital heart disease or Eisenmenger's syndrome.

Design: Retrospective study.

Setting: Tertiary cardiology referral centre.