[Proposal of a common model for informed consent for interventional procedures in congenital heart disease patients]. / Proposta di un modello comune al consenso informato per le procedure di emodinamica nel cardiopatico congenito.

BACKGROUND: A multidisciplinary study group involving physicians and jurists was established to review and approve an informed consent about the most frequent interventional procedures for congenital heart diseases. METHODS: The authors worked together with representatives of the Italian Society of Pediatric Cardiology and Congenital Heart Disease (SICP) Council and Jurist's expert in the field of health case-law. The final draft was shared with the major Italian centers involved in congenital interventional procedures and with AICCA, the Italian Patients Association of Congenital Heart Diseases - Adults and Children. RESULTS: At the end of this review process, a final informed consent form was developed for the most frequent procedures performed in our catheterization laboratories. All of them consist of two parts: a general statement and a procedure-related one. CONCLUSIONS: The work performed by this multidisciplinary study group, under the supervision of the SICP, resulted in a new dedicated informed consent about interventional procedures in the field of congenital cardiology, taking into account the new legal requests. This informed consent is intended to be both a document that can be used as such and a document from which to derive a specific document for each center. We believe that using similar informed consents in all Congenital Heart Disease Centers or at least have informed consents all inspired by the same setting, could be a further improvement in taking care of the patients and their families.

Incidental diagnosis and therapeutic approach of an iatrogenic intra-parenchymal pulmonary intercostal artery pseudoaneurysm: a case report.

Intercostal artery pseudoaneurysms (IAP) are extremely rare but its sudden rupture represents a life-threatening complication. We report an unusual case of a late intercostal artery pseudoaneurysm, after a video-assisted thoracoscopic surgery, presenting as a large intra-parenchymal lung mass. Diagnosis was made by biphasic computed tomography angiography and contrast-enhanced pulsed-wave doppler ultrasound. To minimize the risk of aneurysm bleeding immediate angiographic embolization was planned and successfully performed.IAP should be considered in presence of lung mass in patients who have previously undergone an intervention with intercostal approach to prevent life-threatening complications.

Evaluation of right ventricular function in adults with congenital heart defects.

The right ventricle (RV) is of lesser importance in acquired heart disease, but its role is of increasing importance in congenital heart disease (CHD). Despite major progress being made, precise measurements of the RV are challenging because of its peculiar anatomical structure that is not adaptable to any planar geometrical assumption. This is particularly true in adult patients with CHD where the RV shape eludes any standardization, it may assume various morphologies, and its modality of contraction depends on previous surgical treatment and/or pathophysiological conditions. However, reliable and repeatable quantification of RV dimensions and function for these patients are essential to provide appropriate timing for intervention to optimize outcomes. In this population, echocardiographic evaluation should not be limited to an observational and subjective functional assessment of the RV but must provide quantitative values repeatable and clinically reliable to help the decision-making process. The aim of this review was to discuss the echocardiographic approach to the RV in adult patients with CHD in general and in specific cases of pressure or volume overload.

Cardioembolic stroke: who is the guilty?

A young woman was referred following a transient ischemic attack due to suspect patent foramen ovale. At the diagnostic workup a spontaneous mild right-to-left atrial shunt owing to fenestrated aneurysmal septum was found. However, also a large arteriovenous malformation of the left lung was also imaged. At interventional cardiac catheterization, both malformations were closed using an Amplatzer Cribriform occluding device and a Amplatzer Vascular Plug II, respectively. In conclusion, patent foramen ovale is just one of the potential causes of cardioembolic stroke and a thorough diagnostic workup is mandatory after a cryptogenic stroke to rule out any additional source of paradoxical embolism.

Pulmonary vasoreactivity predicts long-term outcome in patients with Eisenmenger syndrome receiving bosentan therapy.

BACKGROUND: Vasoreactivity testing is recommended in the management of pulmonary arterial hypertension (PAH), but its clinical relevance in congenital heart disease (CHD)-associated PAH has not been established. OBJECTIVE: To determine whether residual pulmonary vascular responsiveness to intravenous. epoprostenol is predictive of clinical outcome in patients with CHD-PAH and Eisenmenger syndrome. METHODS AND RESULTS: A diagnostic right heart catheterisation with reversibility testing using epoprostenol infusion was performed in 38 consecutive patients with CHD-PAH and Eisenmenger syndrome. Patients were treated with bosentan and were assessed every 3 months. Clinical worsening was defined as death from any cause, heart-lung or lung transplantation (or on the waiting list for this procedure), hospitalisation for PAH, or symptom exacerbation defined as a > or =20% decrease in the 6 min walking distance on two consecutive tests, an increase in WHO functional class, or worsening right heart failure. The mean follow-up was 33+/-17 months. Sixteen patients showed clinical worsening. Although they did not differ from the other patients in their baseline exercise capacity, haemodynamic characteristics and underlying CHD, pulmonary vascular resistance index (PVRi) was less reversible (DeltaPVRi 29+/-21 vs 52+/-14%, p=0.0003). At univariate analysis, systemic vascular resistance, PVRi and DeltaPVRi were significant predictors of clinical worsening. At multivariate Cox proportional hazards regression model, DeltaPVRi was found to be the only independent predictor of clinical worsening (HR=0.973, 95% CI 0.95 to 0.99; p=0.01). DeltaPVRi > or =25% had a positive and negative predictive value for clinical worsening of 100% and 75.9%, respectively. CONCLUSION: Pulmonary vasoreactivity is a significant predictor of clinical worsening in patients with CHD-PAH.