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.

[Intrabronchial foreign body in adults: usefulness of computerized tomography]. / Cuerpo extraño intrabronquial en adultos: utilidad de la tomografía computarizada.

The diagnosis of intrabronchial solid foreign body is recorded less often in adults than in children and becomes increasingly difficult to make as time passes after aspiration. Three cases of intrabronchial foreign body in adults with no history of loss of consciousness are reported. Clinical and radiological features are discussed, with special mention of the diagnostic role of computed tomography, a technique that has not often been mentioned in the literature on foreign body aspiration, but that is more sensitive and specific than the simple X-ray. In patients with symptoms inconsistent with X-ray images, computed tomography can be a useful, noninvasive technique for guiding diagnosis and assessing the need for bronchoscopy.

[Active pulmonary tuberculosis in young patients: current radiological presentation forms]. / Tuberculosis pulmonar activa en el paciente joven: formas de presentación radiológica actual.

The clinical histories and thorax X-rays of 91 patients aged between 15 and 25 years who were admitted at the Hospital Royo Villanova, Zaragoza from 1983 to 1987 because of active pulmonary tuberculosis, were reviewed in order to study the radiological presentation of pulmonary tuberculosis in young patients and examine if there were any differences with other age groups. We highlight from the results: 1) The male/female ratio was 1.5/1.2) There were no differences in the radiological presentation between this group and older subjects, 3) The radiological localization of the lesions was the classical one, 4) The mean clinical evolution time before diagnosis was made, was of three months, 5) A longer evolution time was related to a greater lesion extension. We highlight that a prompt X-ray contributes to the early diagnosis of pulmonary tuberculosis in young patients avoiding delays in diagnosis and treatment.

Two-dimensional echocardiographic examination of mitral valve abnormalities associated with coarctation of the aorta.

Coarctation of the aorta is frequently associated with left ventricular inflow tract abnormalities that may be difficult to detect even at cardiac catheterization. This study involved patients with coarctation who underwent comprehensive two-dimensional echocardiographic investigations emphasizing visualization of the mitral valve and its apparatus. Of the 56 patients studied, 23 had completely normal study results, while in 33 (59%) results showed abnormalities of the mitral complex. The latter were divided into two groups: those having major mitral abnormalities (n = 12) and those with minor anomalies of the valve and apparatus (n = 21). The first group included patients with a supravalve stenosing ring, congenital mitral stenosis, mitral valve prolapse, and parachute mitral valve. Minor anomalies were classified as abnormalities of the papillary muscles, chordae tendineae, or combinations of both. No mitral malformations were found in a control group of patients. We conclude that two-dimensional echocardiography is a sensitive and accurate noninvasive method for assessing either significant or subtle forms of left ventricular inflow disease in patients with aortic coarctation. Its use is recommended for the acute medical and surgical management of these patients as well as for their long-term follow-up evaluation.

A rare form of isolated interrupted aortic arch: the value of two-dimensional echocardiography in the precatheterization evaluation.

Preoperative evaluation of a 12-year-old girl, previously diagnosed as having a coarctation of the aorta, revealed a rare form of isolated interruption of the aortic arch. Two-dimensional echocardiography played an important role in the delineation of the defect and significantly reduced the need for invasive techniques. It helped to anticipate problems in reaching left-sided structures at cardiac catheterization, saving time and unnecessary trauma. This technique also ruled out associated intracardiac anomalies that were not detailed at cardiac catheterization.