Management and outcomes in chronic thromboembolic pulmonary hypertension: From expert centers to a nationwide perspective.

BACKGROUND: The Spanish "Registry of Pulmonary Arterial Hypertension" (REHAP), started in 2007, includes chronic thromboembolic hypertension (CTEPH) patients. Based on data provided by this registry and retrospective data from patients diagnosed during 2006 (≤ 12 months since the registry was created), clinical management and long-term outcomes of CTEPH patients are analyzed nationwide for the first time in a scenario of a decentralized organization model of CTEPH management. METHODS AND RESULTS: A total of 391 patients (median [Q1:Q3] age 63.7 [48.0;73.3] years, 58% females) with CTEPH included during the period January 1, 2006-December 31, 2013 in the REHAP registry were analyzed. Rate of pulmonary endarterectomy (PEA) was 31.2%, and highly asymmetric among centers: rate was 47.9% at two centers designated as CTEPH expert centers, while it was 4.6% in other centers. Among patients not undergoing PEA, 82% were treated with therapies licensed for pulmonary arterial hypertension (PAH). Five-year survival rate was 86.3% for PEA patients, and 64.9% for non-PEA patients. Among non-PEA patients, presenting proximal lesions (42% of non-referred patients) was associated with a 3-fold increase in mortality. PEA patients achieved significantly better hemodynamic and clinical outcomes at one-year follow-up compared to non-PEA patients. Patients not being referred for PEA assessment were older and had a worse functional capacity. Older age was the most deterrent factor for non-operability. CONCLUSION: Despite the increase in diagnosis and expertise in PEA-specialized centers, an important percentage of patients do not benefit of PEA in a decentralized organization model of CTEPH management.

[Effectiveness of exercise echocardiography in the diagnosis of left bundle branch block. A case report]. / Eficacia de la ecocardiografía de esfuerzo en el diagnóstico del bloqueo de rama izquierda doloroso. A propósito de un caso.

A left bundle branch block is very often suggestive of cardiac diseases. Left bundle branch block is also present with angina in patients with normal coronary arteries. We report a case of a man with a left bundle branch block in which the most effective, rapid and economic method of diagnosis was exercise echocardiography. Different diagnostic techniques are discussed and compared.

[Neonatal endocarditis due to staphylococcus aureus as a complication of neonatal sepsis]. / Endocarditis neonatal por Staphylococcus aureus como complicación de sepsis neonatal.

We present the case of a newborn with bacterial endocarditis with mitral valve involvement as a complication of late-onset sepsis due to Staphylococcus aureus with associated pyelonephritis and meningitis. The diagnosis was confirmed by echocardiogram and blood culture with growth of S. aureus. Treatment was medical and surgical. Neonatal bacterial endocarditis is extremely difficult to diagnose. The signs and symptoms are usually nonspecific and cannot be distinguished from those of sepsis or congenital heart disease. Consequently, a high degree of suspicion is needed for the early diagnosis of this condition. Echocardiography should be performed in children who present sepsis and heart murmur and even in those with staphylococcemia (sepsis due to S. aureus) without associated heart murmur. This investigation enables an early diagnosis of endocarditis to be made and appropriate treatment to be given without having to wait for the development of signs and symptoms that frequently go undetected.