Incremental value of right atrial strain for early diagnosis of hemodynamic deterioration in pulmonary hypertension: a new noninvasive tool for a more comprehensive diagnostic paradigma.

AIMS: Increased right atrial size is related to adverse prognosis in pulmonary hypertension. The potential incremental value of right atrial function assessment is still unclear. We tested the relationship between right atrial two-dimensional speckle-tracking echocardiography impairment and hemodynamic, functional and clinical deterioration in patients with pulmonary hypertension. METHODS: We prospectively evaluated 36 patients (27 female, 9 male; mean age 68 ±â€Š13 years) with suspected pulmonary hypertension undergoing right heart catheterization and 16 matched controls. All patients underwent baseline evaluation by New York Heart Association functional class, 6-min walking test, brain natriuretic peptide (BNP), and standard two-dimensional echocardiography in less than 48 h of right heart catheterization. Right atrial two-dimensional speckle-tracking echocardiography was assessed by averaging all segments in standard four-chamber apical view. RESULTS: Right atrial global integral strain was significantly lower in patients compared with controls (11.40 ±â€Š5.22% vs. 25.72 ±â€Š5.95 P < 0.001). Moreover, right atrial global strain, but not right atrial area or volume, was correlated with invasively measured cardiac index (CI) (r = 0.72; P < 0.0001) and pulmonary vascular resistances in all patients, even though stronger in subjects with precapillary pulmonary hypertension (r = -0.42, P = 0.018; r = -0.54, P = 0.007 respectively; P = 0.007). It was also correlated with New York Heart Association (P = 0.027), BNP (P = 0.002), and 6-min walking test (P = 0.006). After multivariate analysis including right atrial volume, tricuspid annular plane systolic excursion, left atrial strain, and BNP, right atrial global strain showed the strongest correlation with CI. Area under the curve optimal cutoff for predicting CI at least 2.4 l/min/m was 17% (area under the curve: 0.83, sensitivity: 90%, specificity: 54%). CONCLUSION: Right atrial global strain can identify right atrial functional impairment before structural changes and may be implemented in a comprehensive, noninvasive right heart assessment for diagnosis and follow-up of pulmonary hypertension patients.

[Preliminary results and future perspectives of the Piedmont Adult Congenital Heart Disease Registry]. / Risultati iniziali e prospettive future del Registro Piemontese delle Cardiopatie Congenite dell'Adulto.

BACKGROUND: Adults with congenital heart disease are a relatively new population that progressively increases in size and complexity. In Italy, there are no accurate data concerning the distribution of congenital defects and the long-term outcome relating to both congenital heart disease per se and comorbidities, due to the aging process. METHODS: The Piedmont Adult Congenital Heart Disease Registry has been designed to investigate these aspects and to support a high quality healthcare development for grown-up congenital heart patients. Within 2 years, 459 consecutive patients routinely followed in 10 divisions of cardiology in Piedmont were included in the project. Electronic dedicated software has supported data collection. RESULTS: Mean age of patients is 35 ± 16 years. Septal defects are the most common type of congenital heart disease (35.3%). At baseline evaluation, 71.7% of patients reported a previous surgical and/or percutaneous treatment and 6.3% an electrophysiological procedure. Freedom from intervention is 44%, 81% and 56% at the age of 18, 30 and 45 years, respectively. Patients who had a treatment during infancy show a better intervention-free survival (p=0.038) compared with patients treated during adulthood. Despite the majority of the population had an almost preserved functional status, 27.5% had ≥1 long-term sequelae (arrhythmias 27.5%; pulmonary hypertension 5.2%; neurological problems 4.1%; cyanosis 4.8%; liver dysfunction 2.4%; enteropathy 2.4%; lung disease 2.2%). During 2 years of follow-up, the estimated mortality rate is 0.88%. CONCLUSIONS: Adults with congenital heart disease are a heterogeneous population of relatively young patients with relevant clinical and social problems. The late sequelae related to both the underlying heart disease and the advancing age require continuous monitoring and lifelong specialized care.

Prevalence of undiagnosed chronic thromboembolic pulmonary hypertension after pulmonary embolism.

Chronic thromboembolic pulmonary hypertension is associated with adverse prognosis. Early diagnosis is important to better identify patients who would benefit from a well established therapeutic strategy. The purpose of our study was to evaluate long-term incidence of undiagnosed chronic thromboembolic pulmonary hypertension after acute pulmonary embolism and the utility of a long-term follow-up including an echocardiographic-based screening programme to early detect this disease. We evaluated retrospectively 282 patients discharged from the 'Maggiore della Carità' Hospital, Università del Piemonte Orientale, Novara, Italy, with diagnosis of acute pulmonary embolism between November 2006 and October 2009. One hundred and eleven patients underwent a clinical late echocardiographic screening programme after the acute event. Patients with suspected pulmonary hypertension based on echocardiographic evidence of systolic pulmonary artery pressure of at least 40  mmHg underwent complete work-up for chronic thromboembolic pulmonary hypertension assessment, including ventilation-perfusion lung scintigraphy and right heart catheterization. One hundred and eleven patients were included in the study. Pulmonary hypertension was suspected in 15 patients; five patients had chronic thromboembolic pulmonary hypertension confirmed by ventilation-perfusion lung scintigraphy, right heart catheterization and pulmonary angiography. Two patients with clinical class functionally advanced underwent surgical pulmonary endarterectomy and two asymptomatic patients underwent medical treatment. The prevalence of undiagnosed chronic thromboembolic pulmonary hypertension was 4.5%. Chronic thromboembolic pulmonary hypertension is a serious disease with a poor prognosis if not treated early. Surgical treatment is decisive. After surgery, the majority of patients have a substantial improvement in their functional status and in haemodynamic variables. Many patients are asymptomatic. Implementation of screening programmes may be helpful for an early diagnosis and early proper therapy.