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.

[Right ventricular cardiomyopathy: a diagnosis to bear in mind]. / Miocardiopatía ventricular derecha: un diagnóstico a tener en cuenta.

Arrhythmogenic right ventricular cardiomyopathy is a entity of unknown etiology, that is pathologically characterized by right ventricular myocardial atrophy and fibroadipous tissue replacement. We present the case of a 65-year-old male patient with the diagnosis of chronic liver disease, whose study lead a diagnosis of Arrhythmogenic right ventricular cardiomyopathy.

Miocardiopatia Ventricular Derecha: un diagnostico a tener en cuenta / Right Ventricular Cardiomyopathy: a diagnosis to bear in mind

La miocardiopatía arritmogénica ventricular derecha es una entidad de etiopatogenia desconocida, que patológicamente se caracteriza por atrofia miocárdica ventricular derecha y sustitución por tejido fibroadiposo. Presentamos un paciente de 65 años de edad diagnosticado de hepatopatía crónica, cuyo estudio llevó al diagnóstico de miocardiopatía arritmogénica ventricular derecha. (AU)

[Infectious endocarditis of prosthetic valves due to Staphylococcus capitis: a new case]. / Endocarditis infecciosa sobre válvulas protésicas causada por Staphylococcus capitis: un nuevo caso.

We present the case of a male patient with aortic and mitral valve bioprostheses who developed infectious endocarditis due to Staphylococcus capitis, which has recently been described as an agent producing infectious endocarditis in native and prosthetic cardiac valves. The patient's course evolved unfavorably, despite specific antibiotic treatment, leading to the surgical replacement of the valve, which completely resolved the problem. This case points out that, although rare, in infectious endocarditis due to Staphylococcus capitis its pathogenicity is significant.

[The adult respiratory distress syndrome as a complication of the APSAC treatment of acute myocardial infarct. Apropos a case]. / Síndrome de distrés respiratorio del adulto como complicación del tratamiento del infarto agudo de miocardio con APSAC. A propósito de un caso.

Thrombolytic therapy has shown to be effective in acute myocardial infarction, and its benefits on left ventricular function and later survival are well known. However it is not a therapy free of complications and side effects. Allergic reactions, anafilaxia, hypotension, and several kinds of hemorrhages have been reported. Adult respiratory distress syndrome after streptokinase administration has been also described, and one case, recently communicated, after APSAC therapy. We present the case of a male with acute myocardial infarction who developed adult respiratory distress syndrome after APSAC therapy, with different outcome than the first case published in the literature. Finally, we discussed the mechanisms by means these drugs can produce such a complication.

[Acute myocardial infarct in a cocaine-addicted young man]. / Infarto de miocardio agudo en un joven cocainómano.

We present a case of acute myocardial infarction in a 22 year old cocaine user. The absence of coronary artery stenotic lesions, as was seen in the coronary arteriography, and the absence of personal past cardiovascular or family history, suggest a cocaine-induced coronary spasm as responsible for the acute myocardial event.

[A-V conduction block at His bundle level; as the cause of syncopal crisis in patients with normal ECG (author's transl)]. / Bloqueos A-V completos intermitentes de localización intrahisiana, como causa de crisis sincopales con ECG de base normal.

The history, clinical evolution and complementary studies used to identify the diagnostic etiology of syncopal crisis in a series of 5 patients are presented. The crisis consisted of advanced disturbances of the A-V conduction at the level of the His bundle. It is pointed out that although the evolution of the disturbance was intermittent, the surface ECG was normal and neither A-V block nor broadening of the QRS complex were seen. The importance of continued monitorization, whether as out-patients (Holter system) or, in some cases in an Intensive Care Unit, as well as the need to practice electrophysiologic studies in all these cases as the only way of establishing a firm diagnosis, are underlined. The fact that these disturbances do not always lead to an advanced blockage of A-V conduction is discussed as being reminiscent of the prospective and retrospective studies in the similar case of bi-fascicle block.