The present guideline requires formal dissemination to relevant target user groups, the development of tools for implementation into routine clinical practice and formal evaluation of the impact of the guideline on the quality of care of CTEPH patients. Moreover, the guideline will be updated periodically to reflect new evidence or clinical approaches.

A ventricular septal aneurysm (VSA) is a rare cardiac anomaly, and an accurate statistic of its prevalence has not been reported in the literature. True incidence is likely underestimated as most patients are thought to be asymptomatic. As a result, most VSAs are discovered incidentally on echocardiography, during angiography, or at autopsy. Potential complications include rupture, bacterial endocarditis, right ventricular outflow tract obstruction, and thromboembolic disease. It has been proposed that VSAs occur in association with ventricular septal defects (VSDs) and other congenital cardiac abnormalities. It is uncommon for a VSA to exist in the absence of a known prior ventricular septal defect. We present two cases, each highlighting an incidental intact aneurysm involving the membranous interventricular septum. We discuss the contrast in the two patients with regard to their age, accompanying cardiac anomalies and cardiovascular fitness. Clinical implications of the condition are reviewed.

BACKGROUND: Congenital heart disease (CHD) with systemic-topulmonary shunting is associated with pulmonary arterial hypertension (PAH). There are similar clinical and pathophysiological features between CHD with shunt-associated PAH and idiopathic PAH. Endothelin-receptor antagonists (ERAs) are oral medications that improve pulmonary hemodynamics, symptoms and functional capacity in many PAH patients. However, the role of ERAs in CHD with shunt-associated PAH is unclear. METHODS: MEDLINE, EMBASE and the Cumulative Index of Nursing and Allied Health Literature (CINAHL) databases were searched for articles published from 1966 through September 2006, as well as bibliographies of all retrieved papers. All published English-language studies of adult CHD patients with shunt-associated PAH treated with ERAs were reviewed for clinical, functional and hemodynamic outcomes. RESULTS: Ten studies of 174 adult CHD subjects with shunt-associated PAH were identified. Other than one placebo-controlled, randomized clinical trial, all studies were open-label, uncontrolled observational trials. Subjects were treated with the ERA bosentan for a mean (± SD) of 9±7 months. Nine studies reported improved World Health Organization (WHO) modification of the New York Heart Association functional class, with 95 of 164 subjects (58%) improving by at least one functional class. The 6 min walk distance improved in all eight studies in which it was assessed. Bosentan was generally well tolerated; 2.3% of subjects withdrew because of elevated liver enzymes. Two patients with WHO functional class IV PAH died during bosentan therapy. CONCLUSION: Treatment of CHD patients with shunt-associated PAH with the ERA bosentan is associated with an improvement in functional class and objectively measured exercise capacity. The consistency of the uncontrolled data and the positive results of a single randomized clinical trial suggest a role for ERA therapy in CHD patients with shunt-associated PAH. Caution is suggested when considering bosentan therapy for CHD patients with WHO functional class IV PAH.

Some authors have expanded the indication and applied the technique to adult groups. Mishra and colleagues also used a bidirectional Glenn shunt to redirect some of the blood volume into the right pulmonary artery in a patient with endomyocardial fibrosis in whom the blood flow to the right ventricle was restricted [1]. In a similar case where there was right ventricle outflow tract obstruction and right ventricle endomyocardial fibrosis, Anbarasu and coworkers performed a bidirectional Glenn shunt, a De Vega annuloplasty and enlarged the right ventricle outflow tract with a pericardial patch. They obtained a decrease in the blood volume returning to the right ventricle and also managed to correct the right ventricle outflow stenosis [2]. Shu-Chien et al. used a bidirectional Glenn shunt to avoid valve replacement in 4 young drug abusers with tricuspid valve endocarditis. They excised the tricuspid leaflets and reduced the tricuspid annulus to a Z size of − 2 to − 4. In their report, the authors claimed that reducing the tricuspid annulus to a Z size of − 4 was well tolerated without producing stenotic symptoms [5]. The peculiarity of our case compared to the aforementioned ones is that we performed a Glenn shunt in an adult patient with rheumatoid triple valve disease. In the operation we calculated the Z value as − 6 and concluded that the already existing gradient would not decrease after TVR. Therefore, we preserved the normally functioning, disease-free leaflets and diverted 1/3 of the systemic venous return to the right ventricle to the right pulmonary artery. This allowed volume and functions of the right ventricle in our patient to be preserved. Four years after the operation, the patient is still on medical treatment without any problems. A small tricuspid annulus is a serious problem in adults with valvular heart disease. In this group of patients, if the mean pulmonary artery pressure is expected to decrease postoperatively, a palliative bidirectional Glenn shunt should be considered. Despite the advancing technology of bioprostheses, a significant decline in the morbidity and mortality rates of tricuspid diseases has not been obtained yet. Therefore, we believe conservative treatment should be the first choice of treatment for tricuspid pathologies, and tricuspid valve replacement should be reserved for when no treatment modality is left.