IVNC is associated with a broad spectrum of clinical and pathophysiological findings, and the overall natural history and prognosis may be better than previously thought. Adult patients with incidental or familial discovery of IVNC have an encouraging outlook, whereas those who have symptoms of heart failure, a history of sustained ventricular tachycardia or an enlarged left atrium have an unstable course and more severe prognosis.

Echocardiography plays an important role in the diagnosis and follow-up of aortic diseases. Evaluation of the aorta is a routine part of the standard echocardiographic examination. Transthoracic echocardiography (TTE) permits adequate assessment of several aortic segments, particularly the aortic root and proximal ascending aorta. Transoesophageal echocardiography (TOE) overcomes the limitations of TTE in thoracic aorta assessment. TTE and TOE should be used in a complementary manner. Echocardiography is useful for assessing aortic size, biophysical properties, and atherosclerotic involvement of the thoracic aorta. Although TOE is the technique of choice in the diagnosis of aortic dissection, TTE may be used as the initial modality in the emergency setting. Intimal flap in proximal ascending aorta, pericardial effusion/tamponade, and left ventricular function can be easily visualized by TTE. However, a negative TTE does not rule out aortic dissection and other imaging techniques must be considered. TOE should define entry tear location, mechanisms and severity of aortic regurgitation, and true lumen compression. In addition, echocardiography is essential in selecting and monitoring surgical and endovascular treatment and in detecting possible complications. Although other imaging techniques such as computed tomography and magnetic resonance have a greater field of view and may yield complementary information, echocardiography is portable, rapid, accurate, and cost-effective in the diagnosis and follow-up of most aortic diseases.

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† This joint statement, which represents the views of the EACTS and the ESC, was arrived at after careful consideration of the available evidence at the time it was written. Health professionals are encouraged to take the joint statement fully into account when exercising their clinical judgement. The joint statement does not, however, override the individual responsibility of health professionals to make appropriate decisions in the circumstances of the individual patients, in consultation with that patient, and where appropriate and necessary the patient's guardian or carer. It is also the health professional's responsibility to verify the rules and regulations applicable to drugs and devices at the time of prescription.

Endovascular stent-graft repair is less invasive in patients with chronic and acute descending thoracic aortic aneurysm and dissection.

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Background-This large, prospective, multicentric study was performed to analyze the distribution of tricuspid regurgitation velocity (TRV) values during exercise and hypoxia in relatives of patients with idiopathic and familial pulmonary arterial hypertension (PAH) and in healthy control subjects. We tested the hypothesis that relatives of idiopathic/familial PAH patients display an enhanced frequency of hypertensive TRV response to stress and that this response is associated with mutations in the bone morphogenetic protein receptor II (BMPR2) gene. Methods and Results-TRV was estimated by Doppler echocardiography during supine bicycle exercise in normoxia and during 120 minutes of normobaric hypoxia (FIO 2 ϭ12%; Ϸ4500 m) in 291 relatives of 109 PAH patients and in 191 age-matched control subjects. Mean maximal TRVs were significantly higher in PAH relatives during both exercise and hypoxia. During exercise, 10% of control subjects but 31.6% of relatives (PϽ0.0001) exceeded the 90% quantile of mean maximal TRV seen in control subjects. Hypoxia revealed hypertensive TRV in 26% of relatives (Pϭ0.0029). Among control subjects, TRV at rest was not related to age, sex, body mass index, systemic blood pressure, smoking status, or heart rate. Within kindreds identified as harboring deleterious mutations of the BMPR2 gene, a hypertensive TRV response occurred significantly more often compared with those without detected mutations. Conclusions-Pulmonary hypertensive response to exercise and hypoxia in idiopathic/familial PAH relatives appears as a genetic trait with familial clustering, being correlated to but not caused by a BMPR2 mutation. The suitability of this trait to predict manifest PAH development should be addressed in long-term follow-up studies.

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