ABSTRACT
BACKGROUND: Integrated transthoracic and transesophageal echocardiography enables identification and characterization of a quadricuspid aortic valve anomaly. CASE PRESENTATION: A totally asymptomatic 40-year-old white man was referred to our Division of Cardiology after accidental finding of a heart murmur. Transesophageal echocardiography detected a quadricuspid aortic valve characterized by four cusps of equal size and severe aortic valvular regurgitation, without any further anomalies. He underwent a successful aortic valve repair. CONCLUSIONS: Quadricuspid aortic valve anomaly is a rare congenital cardiac defect that can cause progressive valvular complications.
Subject(s)
Aortic Valve Insufficiency/diagnostic imaging , Aortic Valve/diagnostic imaging , Heart Defects, Congenital/diagnostic imaging , Heart Valve Diseases/diagnostic imaging , Adult , Aortic Valve/surgery , Aortic Valve Insufficiency/surgery , Echocardiography, Transesophageal , Heart Defects, Congenital/surgery , Heart Valve Diseases/surgery , Humans , MaleABSTRACT
BACKGROUND: The primary objective of this post hoc analysis was to evaluate clinical outcomes of tadalafil in patients with pulmonary arterial hypertension (PAH) associated with connective tissue disease (CTD-PAH) compared with patients with idiopathic/heritable PAH (I/H-PAH) for primary and key secondary efficacy endpoints, and safety. This analysis included adult patients with CTD-PAH or I/H-PAH who participated in the PHIRST and PHIRST-2 studies. METHODS: Patients were randomized 1:1:1:1:1 to tadalafil (2.5, 10, 20, or 40mg) or placebo in the PHIRST study and the majority of these patients were subsequently assigned 40mg in PHIRST-2. Patients taking 20mg in PHIRST without demonstrating clinical worsening continued on 20mg in PHIRST-2. Outcomes analyzed included 6MWD, WHO-FC, and incidence and time to first occurrence of clinical worsening. Safety was assessed through evaluation of adverse events (AEs), clinical laboratory data, electrocardiograms, and physical examinations. RESULTS: Increased 6MWD in PHIRST was maintained in both CTD-PAH and I/H-PAH subgroups for 52weeks. Patients with CTD-PAH tended to be older, were more likely female, had lower exercise capacity, were more likely to have clinical worsening, and experienced AEs more frequently than patients with I/H-PAH. CONCLUSION: The effect of tadalafil treatment in patients enrolled in both PHIRST studies was detectable for both I/H-PAH and CTD-PAH subgroups. In general, subgroup differences were modest. Patients with CTD-PAH may perform less well than patients with I/H-PAH in safety and efficacy measures in all treatment groups, which is similar to other studies demonstrating a worse prognosis for patients with CTD-PAH.
Subject(s)
Connective Tissue Diseases/complications , Drug-Related Side Effects and Adverse Reactions/diagnosis , Familial Primary Pulmonary Hypertension , Hypertension, Pulmonary , Tadalafil , Dose-Response Relationship, Drug , Drug Monitoring/methods , Electrocardiography/methods , Familial Primary Pulmonary Hypertension/diagnosis , Familial Primary Pulmonary Hypertension/drug therapy , Female , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/drug therapy , Hypertension, Pulmonary/etiology , Male , Middle Aged , Physical Examination/methods , Tadalafil/administration & dosage , Tadalafil/adverse effects , Treatment Outcome , Vasodilator Agents/administration & dosage , Vasodilator Agents/adverse effectsABSTRACT
Pulmonary arterial hypertension is a severe disease with a complex pathogenesis, for which combination therapy is an attractive option.This study aimed to assess the impact of sequential combination therapy on both short-term responses and long-term outcomes in a real-world setting.Patients with idiopathic/heritable pulmonary arterial hypertension, or pulmonary arterial hypertension associated with congenital heart disease or connective tissue disease and who were not meeting treatment goals on either first-line bosentan or sildenafil monotherapy, were given additional sildenafil or bosentan and assessed after 3-4 months. Double combination therapy significantly improved clinical and haemodynamic parameters, independent of aetiology or the order of drug administration. Significant improvements in functional class were observed in patients with idiopathic/heritable pulmonary arterial hypertension. The 1-, 3- and 5-year overall survival estimates were 91%, 69% and 59%, respectively. Patients with pulmonary arterial hypertension associated with connective tissue disease had significantly poorer survival rates compared to other aetiologies (p<0.003).The favourable short-term haemodynamic results and good survival rates, observed in patients receiving both bosentan and sildenafil, supports the use of sequential combination therapy in patients failing on monotherapy in a real-world setting.